ACHD Featured Articles of November 2015

1. Myocardial infarction in grown up patients with congenital heart disease: An emerging high-risk combination.

Sieweke JT, Westhoff-Bleck M, Napp LC, Avsar M, Vogel-Claussen J, Schäfer A, Bauersachs J, Brehm M.

Int J Cardiol. 2015 Oct 9;203:138-140. doi: 10.1016/j.ijcard.2015.10.020. [Epub ahead of print] No abstract available.

PMID: 26512828

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Moe_Tabitha-WEBComment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: Double outlet LV is a rare ventriculoarterial malalignments with a <1% incidence in childhood.  This case encapsulates the challenges of our older adult survivors of CHD.  This is single-ventricle physiology, LV morphology, with severe subvalvar PS.  His initial presentation was with an acute MI, complicated by a post-MI pleural effusion, and a concerning pericardial effusion secondary to free-wall rupture. Mortality in this scenario in non-congenital patients approaches 50%, and perhaps even higher with single-ventricle physiology. These cases are exceedingly rare, but increasing in frequency.  This scenario presents the need for vigilance in our repaired congenital heart disease patients for risk factor modification leading to acquired heart disease.  This also underlies the need for education of our adult cardiology colleagues in resources for congenital heart disease, anatomy, and physiology. As more and more of these types of CHD and STEMI cases accumulate, it will become progressively more clear what the risks are of acquired heart disease in the setting of underlying CHD.

Take Home point: Cardiovascular risks in patients with congenital heart disease should be appropriately modified. ACS remains a real risk in patients with CHD.


2. Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions.

Blok IM, van Riel AC, Mulder BJ, Bouma BJ.

Expert Rev Cardiovasc Ther. 2015 Oct 16:1-16. [Epub ahead of print]

PMID: 26473378

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This is an excellent article evaluating the state of our current understanding of pulmonary arterial hypertension secondary to congenital heart disease.  It is well known that the development of frank pulmonary hypertension is associated with marked increase in morbidity and mortality.  Over the past decade there has been a progressive emphasis on more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.  Despite aggressive management PAH-CHD has a significant impact on morbidity and mortality. PAH many decades after shunt closure remains an under-recognized phenomenon.  Prostacyclins are well-documented as effective and are reasonable first-line therapy for such cases.  Endothelin receptor antagonists have a progressively more established and proven place in management. Unfortunately, patients with complex congenital heart disease are often excluded from trials, and the after-market application of drug forms is variably implemented and reported. BREATHE-5 continues to be the landmark trial of ERA in CHD. Combination therapy can be used, but it should be appreciated that the need to add an additional agent is linearly related to an increase in both morbidity and mortality as the seeming protective effect on morbidity of CHD is lost with the addition of a second agent.

Take Home point: 1. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.


3. 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients.

Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O’Leary PW, Driscoll DJ, Cetta F.

J Am Coll Cardiol. 2015 Oct 13;66(15):1700-10. doi: 10.1016/j.jacc.2015.07.065.

PMID: 26449141

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This may be the landmark paper for the calendar year 2015.  This is a typical Mayo data evaluation of a massive amount of data (>1000 patients) that is expertly evaluated, statistically analyzed and reported.  The limitations are that this is a single-center study, and this data may not apply to all populations.  However, since reading this paper I have quoted numbers out of it no less than 5 times to patients, to families, and to my pediatric colleagues.  The important take-aways for your immediate use are that the 10-year survival post-Fontan is 74%, the 20-year is 61%, and the 30-year is 43%.  The most common challenges are (no surprise) the life-time development of PLE and arrhythmias. The survival numbers following the development of PLE are equally valuable, and quotable: 5-year 50%, 10-year 35%, and 20 year 19%. Many of these patients continued to be follow at institutions outside the Mayo system.  Their oldest Fontan was completed at age 53, with the youngest occurring as early as 7 months, reflecting the pendulum swing of timing of Fontan completion. Interestingly they only identify 24 patients with HLHS, so this young cohort, of primarily RV morphology is quite underrepresented, and this data may not apply to them. Longer follow up is needed. The oldest documented Fontan survivor is 67 and underwent Fontan completion at age 39, as this is a question I frequently get from patients it is an excellent number to be able to pull out for them as well.  Fenestration does not seem to correlate with increased overall survival.  There are only 2 documented cases of hepatocellular carcinoma, which is reassuring for all the concern arising from chronic hepatopathy. However, 2:1000 is infinitely higher odds for development of HCC than we would prefer. Additionally, their data suggest that the 30-year freedom from arrhythmia is only 24%, consistent with the Boston data, but continues to emphasize the need for EP support in the chronic management of these patients. The majority of arrhythmia patients had atrial flutter or fibrillation.

Take Home point: The 10-year survival post-Fontan is 74%, the 20-year is 61%, and the 30-year is 43%. However, caution in extrapolating this is primarily in a group of older morphologically left ventricle patients.


4. Effect of Sildenafil on Pressure-Volume Loop Measures of Ventricular Function in Fontan Patients.

Butts RJ, Chowdhury SM, Baker GH, Bandisode V, Savage AJ, Atz AM.

Pediatr Cardiol. 2015 Sep 26. [Epub ahead of print]

PMID: 26409473

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: The objective of the study was to attempt to understand the acute effect of sildenafil on the pressure-volume loop in the cath lab.  There is conflicting evidence and variable use of phosphodiesterase agents in our Fontan population and the short and long-term benefits are variable. This is a small, but very well-designed trial by one of the giants in Fontan physiology and pulmonary hypertension.  Only 19 patients were tested in this randomized, double-blind trial.  The outcomes suggest that there is immediate ventriculoarterial coupling changes.  However, end-systolic elastance did not improve with sildenafil, but did with dobutamine.  It is imperative to continue to study these patients as there is a progressive decrease in exercise capacity and a correlated decrease in quality of life that we may be able to affect long-term with PDE-5, but that is not born out in the immediate term application with single-dose sildenafil. There may be long-term improvements in end-elastance, that cannot be completely captured with a pressure-volume loop. Mentally tuck this study away, there are more trials on the horizon.

Take Home point: PDE5i agents are routinely used in Fontan patients and the potential long term improvements in end-elastance can not be determined with single-dose immediate result testing.


5. Diagnostic value of the six-minute walk test (6MWT) in grown-up congenital heart disease (GUCH): Comparison with clinical status and functional exercise capacity. Kehmeier ES, Sommer MH, Galonska A, Zeus T, Verde P, Kelm M.

Int J Cardiol. 2015 Oct 21;203:90-97. doi: 10.1016/j.ijcard.2015.10.074. [Epub ahead of print]

PMID: 26498869

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: Exercise testing of our older patients with CHD is a critical factor in our ongoing evaluation of these patients.  A quick snapshot, with low cost, and high significance would be valuable.  Cardiometabolic stress testing may be ideal, but this test may only be available at larger centers, requires a few hours to complete, patient preparation, and an experienced cardiac physiologist to interpret the complex data.  The ready availability and simple performance standards of a 6 MWT has immense appeal to the clinic cardiologist, and the study can be performed either as a sole testing mode or as interval testing between routinely scheduled cardiopulmonary stress testing.  6MWT has already been validated in heart failure, pulmonary hypertension, other lung diseases, and dilated cardiomyopathy.  The 6MWT demonstrates close correlation to peak VO2 and is an excellent predictor of decreased peak VO2 with a breakpoint of <15.5 mL/kg/min. There is excellent test and retest reliability.  The shortest 6MWD of 280 meters is consistently in Eisenmenger patients. The 6MWT is a promising tool in evaluating exercise capacity and functional status in our ACHD/GUCH patients. A 6 MWT >350 meters is consistent with a VO2 max >30% for CHD diagnosis.

Take Home point: The 6 MWD is a good surrogate for VO2 max for our older ACHD patients. Consider performing 6 MWD in the interval visits between CPET’s.


6. Right heart failure in pregnant women with cyanotic congenital heart disease – The good, the bad and the ugly.

Zengin E, Sinning C, Schrage B, Mueller GC, Klose H, Sachweh J, Goepfert M, Hueneke B, Blankenberg S, Kozlik-Feldmann R.

Int J Cardiol. 2015 Oct 9;202:773-775. doi: 10.1016/j.ijcard.2015.10.036. [Epub ahead of print] No abstract available.

PMID: 26474467

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This is a really nice German study evaluating a complex group of patients with pregnancy and congenital heart disease.  There are well-understood risks for both the mother and child with pulmonary arterial hypertension.  This is a short case series of patients with pulmonary hypertension.  One with an unrestrictive VSD, one with unrepaired truncus Type I, and one with TOF palliated with a Waterston up till age 7 at the time of complete repair.  Each of these patients would be considered high risk by any risk score calculator including WHO, ZAHARA, and CARPREG. Of the three, the lowest pre-pregnancy risk patient experienced an immensely complicated outcome with a premature delivery and dramatically progressive RV failure.  There was no long-term  outcomes reported for these PAH-CHD patients.  Although, no one would counsel these patients to plan a pregnancy, they do present, and it is important to understand their management and outcomes for counseling.

Take Home point: Patients with complex congenital heart disease who would not be counseled to become pregnant still do, and their outcomes are widely variable, difficult to predict, and require close monitoring at an integrated center.


7. A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease.

Strange G, Rose M, Kermeen F, O’Donnell C, Keogh A, Kotlyar E, Grigg L, Bullock A, Disney P, Dwyer N, Whitford H, Tanous D, Frampton C, Weintraub R, Celermajer DS.

Intern Med J. 2015 Sep;45(9):944-50. doi: 10.1111/imj.12821.

PMID: 26011062

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Comment from guest editor Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This is a very small registry in Australia and New Zealand comprising of 370 patients demonstrating a symbiotic relationship with industry.  It is well-known that ~5% of patients with CHD ultimately develop PAH.  60% enrolled were female, and all-cause mortality was 12% at 5 years, and 31% at 15 years. Only 117/370 underwent right heart catheterization (RHC), and I would typically consider this standard of care in the US.  In fact many American  insurance companies require PAH to be documented by RHC prior to initiating advanced PAH therapies.  43% of their cohort was anticoagulated, and these patients did not inherently have a thrombotic diagnosis, and this therapy remains controversial. 40% received diuretics, and 16% were prescribed digoxin. We note that these 370 patients represent only 10% of the PAH-CHD patients that statistically should be expected in this population.  These unidentified patients are at high risk for adverse outcomes.  Additionally, even in the captured cohort there is a delay in diagnosis of PAH by up to 6 years.  The largest causative factor is related to the gap in care in the transition between pediatric and adult CHD centers.  Ongoing efforts to assure appropriate transition from pediatric to adult care remains vital.

Take Home point: Patients with simple and moderate complexity congenital heart disease need to be appropriately transitioned to avoid interval development and decompensation of comorbidities like PAH-CHD.


8. Stroke in Adults With Congenital Heart Disease: Incidence, Cumulative Risk and Predictors.

Lanz J, Brophy JM, Therrien J, Kaouache M, Guo L, Marelli AJ.

Circulation. 2015 Nov 23. pii: CIRCULATIONAHA.115.011241. [Epub ahead of print]

PMID: 26597113

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Mehul Patel photoComment from Dr. Mehul Patel (MSU, MI) section editor of Congenital Heart Journal Watch:  In this landmark study, the authors retrospectively evaluated the incidence, cumulative risk, and predictors of stroke in 29,638 Quebec patients with adult congenital heart disease. While there is an overall decline in stroke incidence and mortality in the general population over the past decades due to major improvement in population health in both genders and all race and age groups, the risk in the ACHD population remains high.

The cumulative risk of ischemic stroke estimated up to age 64 years was 6.1%. Compared with rates reported for the general Quebec population, age-sex standardized incidence rates of ischemic stroke were 9 to 12 times higher below age 55 years and 2 to 4 times higher in the age group 55 to 64 years; hemorrhagic stroke rates were 5 to 6 times (age <55 years) and 2 to 3 times higher.  The strongest predictors of ischemic stroke were heart failure, followed by diabetes mellitus and recent myocardial infarction.

Take home points:

  1. Among patients with adult congenital heart disease, 1 in 11 men and 1 in 15 women experienced a stroke between ages 18 and 64 years.
  2. Stroke incidence was considerably higher than in the general population, especially at a younger age.
  3. The most important predictors of ischemic stroke were heart failure, diabetes mellitus, and recent myocardial infarction. Additional research is required to see whether advances in the management of adult congenital heart disease may reduce this substantial stroke rate.

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CHD Electophysiology Featured Articles of November 2015

1. Outcome in young patients with isolated complete atrioventricular block and permanent pacemaker treatment: A nationwide study of 127 patients.

Eliasson H, Sonesson SE, Salomonsson S, Skog A, Wahren-Herlenius M, Gadler F; Swedish Congenital Heart Block Study Group.

Heart Rhythm. 2015 Nov;12(11):2278-84. doi: 10.1016/j.hrthm.2015.06.028. Epub 2015 Jun 18.

PMID: 26096611

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cohen picComment from Dr. Mitchell Cohen (Phoenix), section editor of Congenital Heart Electrophysiology Journal Watch: Congenital AV block (CCAVB) is associated with exposure to maternal lupus autoantibodies whereas isolated AV block tends to be diagnosed later and is not associated with autoantibodies. Most patients with CCAVB do well clinically, however, a subset of patients develop a dilated cardiomyopathy (DCM) and even a few have gone on to require heart transplantation. A number of theories have been put forth regarding why this subset of patients develops a DCM including long-term chronic RV apical pacing, presence of autoantibodies, and an augmented autoantibody load. CCAVB is a very rare condition and as such most studies have so few patients that definitive conclusions regarding causation remain elusive. Dr. Eliasson and his colleagues working within the Swedish Congenital Heart Block Study Group performed a retrospective cohort study of 127 patients (< 18 years of age) with non-surgical AV block, diagnosed prior to age 15 years to understand the factors that might contribute to developing LV dysfunction. Patients were followed for nearly 9 years and approximately 2/3 of the study group were autoantibody positive. Patients diagnosed in the first month of life tended to be autoantibody positive whereas no patient diagnosed with AV block after 5 years of age was antibody positive. Nine patients (8%) developed LV dysfunction, a percentage similar to a multicenter study in 2001 by Udink ten Cate where 6% developed a DCM. In this most recent study, all patients who developed a DCM were diagnosed within the first month of age and all had LV dysfunction prior to receiving their pacemaker. Male predilection was also more common in the small number of LV dysfunction neonates with CCAVB. Alternatively, NO patients with normal LV function developed a DCM regardless of age at presentation. This information is very valuable when counseling families with an in-utero or very early age at diagnosis of AV block. A growing number of clinical electrophysiologists in conjunction with their congenital heart surgeons have moved towards putting the ventricular pacing lead on the LV in the neonate/infant who requires pacing. It is unclear if such a proactive approach to LV pacing can alter the natural history. Since the majority of neonates presenting in the first 30 days of life DO NOT develop a DCM, there are likely other contributing factors that result in the development of LV dysfunction. Ongoing multicenter research trials in this rare condition will be required to answer this complex question.

Take Home Points:

  • The majority of neonates diagnosed with congenital AV block are auto-antibody positive (>90%).
  • Neonates presenting with heart block and LV dysfunction have an increased risk of ongoing LV dysfunction (90%) compared to infants presenting beyond a month of age.
  • Ongoing research will need to best evaluate why some neonates with autoantibodies develop LV dysfunction and others do not.
  • This study was not powered to compare RV apical pacing with LV pacing.


2. Revisiting the challenges of universal screening for long QT syndrome.

Tristani-Firouzi M.

J Electrocardiol. 2015 Nov-Dec;48(6):1053-7. doi: 10.1016/j.jelectrocard.2015.08.024. Epub 2015 Aug 12.

PMID: 26355713

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Comment from Dr. Mitchell Cohen (Phoenix), section editor of Congenital Heart Electrophysiology Journal Watch: Dr. Tristani from Primary Children’s Hospital in Utah has written an excellent review article that looks at universal screening for LQT in the context of how other rare conditions have adjudicated newborn screening As Dr. Tristani points out, universal screening for a disorder is appropriate if three conditions are met. “(1) there is detailed information regarding the frequency and natural history of the disorder; (2) the safety and cost-effectiveness of the treatment; and (3) the quality of the screening test.” In long QT syndrome the first and second conditions are known. We are aware that the incidence of LQT is 1:2500-1:5000 and that untreated LQT carries a 15% incidence of sudden death while in patients with LQT B-blocker treatment reduces the incidence of SCD to < 1%. The problem with universal screening rests in the 3rd condition: The Quality of the Screening Test. ECG measurements, while still the gold-standard in evaluating patients for LQT, have some inherent flaws; this is probably most true in evaluating the neonate/infant for LQT. In addition, there is no categorical absolute QT measurement that defines an LQT patient. Patients with LQT (genotype positive) may in fact have a normal QT and 15% of the normal population can have a QT that is prolonged. Genetic testing also has its limitations with too numerous to count variants of unknown significance and a prohibitive financial expenditure of the health care dollar. Novel strategies such as QT measurement using root mean square electrocardiography may help reduce some of the false-positives and false-negatives when evaluating a neonatal ECG. This is a well-written article that addresses some of the barriers to universal screening and out of the box thinking that will be required if we are going to move forward in screening for LQT at a national level.

Take Home Points:

  • The incidence of LQT and both the treated and untreated natural history is generally well known.
  • Current ECG screening is fraught with false-positives and false-negatives.
  • Newer ways to calculate a QT, such as root mean square, may eliminate some of the inadequacies that currently exist with standard EKG reading especially in neonates and infants.
  • Ongoing efforts to minimize variants of unknown significance while lowering costs will facilitate a better screening process. However, it is unlikely that in the immediate future current health care expenditures will have routine whole exome sequencing replacing gold-standard EKG reading.
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CHD Interventions Featured Articles of November 2015

1. Selective propensity of bovine jugular vein material to bacterial adhesions: An in-vitro study.

Jalal Z, Galmiche L, Lebeaux D, Villemain O, Brugada G, Patel M, Ghigo JM, Beloin C, Boudjemline Y.

Int J Cardiol. 2015 Nov 1;198:201-5. doi: 10.1016/j.ijcard.2015.07.004. Epub 2015 Jul 6.

PMID: 26173058

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bouddjemlineMehul Patel photoComment from Pr. Younes Boudjemline (Paris) and co-editor Dr. Mehul Patel (MSU, MI), editors of congenital heart disease interventions Journal Watch:  Percutaneous valves are prone to infective endocarditis and may have varied clinical presentations. While Melody valve (stented bovine jugular vein) is considered safe and effective, for unclear reasons, it has an incidence of endocarditis akin to its surgical counterpart, the Contegra valved conduit. Selective bacterial adhesion in-vitro on three valved stents (Melody valve, homemade stents with bovine and porcine pericardium) using Staphylococcus aureus and Streptococcus sanguinis strains was studied with the aid of scanning electron microscope.  Bacterial adhesion was higher on bovine jugular venous wall for S. aureus and on Melody valve leaflets for S. sanguinis in control groups and significantly increased in traumatized Melody valvular leaflets with both bacteria. Bacterial adhesion was lower on bovine pericardial leaflets.

Take home points:

  1. There seems to be abnormal tropism and selective adhesion of S. aureus and S. sanguinis to the healthy Melody valve.
  2. Procedural preparation for Melody® valve implantation resulted in significantly increased bacterial adhesion.
  3. It is possible that traumatic lesions may increase surface roughness by modifying topographical characteristics of the substrate, and thus enable higher microbial adhesion. Further studies may be warranted to prove this.
  4. Bovine pericardium is the substrate in which bacterial adhesion was the lowest.

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CHD Surgery Featured Articles of November 2015

1. Durability of Mitral Valve Repair Performed Before the Age of 5 Years.

Ando M, Takahashi Y.

Circ J. 2015 Nov 13. [Epub ahead of print]

PMID: 26567483 Free Article

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abdulgani picComment from Dr. Hafil B. Abdulgani (Indonesia), section editor of Congenital Heart Surgery Journal Watch: This is a very nice article describing several mitral valve repair techniques in children less than 5 years of age. Due to the fragile nature of the valve in this young population, annuloplasty seems to be the most plausible approach compared to leaflet intervention. The author interestingly utilizes Alfieri’s edge-to-edge valvuloplasty technique, originally described for adult with ischemic mitral regurgitation. Techniques performed, in 51 patients aged younger than 5 years, were commissural annuloplasty, Alfieri’s edge-to-edge, commissural closure, closure of the accessory cleft or hole, sliding leaflet, artificial chordal placement and chordal shortening. There were 3 deaths. The postoperative degree of mitral regurgitation was mild or less in 41 patients (80.4%); 9 patients required reoperation for mitral regurgitation or stenosis. Freedom from reoperation for patients with isolated mitral regurgitation and those with other congenital heart disease at 10 years was 91.7±68.0% and 68.4±9.4%, respectively. Actuarial survival was 97.0±3.0% and 85.1±9.7%, respectively. The authors concluded that excellent survival rates were achieved after mitral valve repair in patients younger than 5 years. The incidence of both reoperation and significant regurgitation was acceptable.

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Figure 1. Schematic of commissural annuloplasty technique. The stitch is essentially placed along the entire commissural leaflet (A). The stitch may be placed more posteriorly to stretch and bring the posterior leaflet towards the anterior leaflet (B).

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 Figure 2. Posterior annuloplasty technique using multiple pledgetted stitches.

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Figure 3. Schematic of sliding leaflet technique. First, the prolapsing segment is resected in a quadrangular fashion. Next, the adjacent leaflet is detached from the annulus for approximately a half-length of the gap (A). The 2 ends of the remaining leaflets are approximated by compression sutures placed on the annulus, and sutured together (B).

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Numbers in parenthesis indicate patients at risk. The percentage was 72.7±8.4 at 1 year and 68.4±9.4, respectively, after 5 years for congenital heart disease, and 91.7±8.0% after 1 year for isolated mitral regurgitation. The P value for this difference (log-rank test) was 0.1363.

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Numbers in parenthesis indicate patients at risk. The percentage was 97.0±3.0 after 0.08 year for congenital heart disease, and 85.1±9.7% after 0.98 year for isolated mitral regurgitation. The P value for this difference (log-rank test) was 0.1693.

Alfieri’s edge-to-edge technique is reported to be effective even for young children. However, the author made every effort to avoid this approach unless the patient has a sufficiently enlarged MV annulus. This is in contrast with the same technique applied for a tricuspid valve (suturing of the anterior and sepal leaflets) and a common atrioventricular valve with a single ventricle (2 bridging leaflets) where unhindered opening of the 2 orifices can be expected.

Take home points:

  1. Excellent survival rates were achieved after mitral valve repair in patients younger than 5 years.
  2. The incidence of both reoperation and significant regurgitation was acceptable.
  3. Alfieri’s edge-to-edge technique is reserved for patient with enlarged MV annulus.


 2. No-React® Injectable BioPulmonic™ valves re-evaluated: discouraging follow-up results.

Grohmann J, Höhn R, Fleck T, Stiller B, Neumann E, Sigler M, Kroll J, Beyersdorf F, Siepe M.

Interact Cardiovasc Thorac Surg. 2015 Nov;21(5):657-65. doi: 10.1093/icvts/ivv214. Epub 2015 Aug 1.

PMID: 26232985 Free Article

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Comment from Dr. Hafil B. Abdulgani (Indonesia), section editor of Congenital Heart Surgery Journal Watch: This is a retrospective analysis, mid-to-long-term follow-up results after implantation of the No-React® Injectable valve. The No-React® Injectible valve was introduced as a modern surgical valve replacement for minimally invasive and even off-pump procedures. The potential advantages were ease of implantation, favourable tissue constitution and a perfect landing zone for a percutaneous valve-in-valve. Although there were no deaths or major morbidity following the redo surgery and interventions, the results were disappointing; i.e. longevity in conjunction with freedom from re-replacement was just 14% after 7 years. The reasons for replacement were severe neointimal reaction of the No-React® valve in 3 patients and imperfect positioning from the initial implantation (‘injection’) in 3 others. Interventional revalvulation and surgical valve exchange were required.

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Figure 1: Replacement-free survival after No-react valve implantation estimated by Kaplan–Meier method.

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Figure 2: A 25 mm No-React® Injectable BioPulmonicTM valve 6 years after implantation (Patient 2): significant reobstruction due to an impressive neointimal built-up; stent-struts anchored deeply (A/B). RVOT- ballooning including left coronary artery angiography simultaneously (C/D). After prestenting (one Covered CP Stent (TM) and one CP Stent (TM) 39 mm, NuMed), revalvulation (Melody TPV, Medtronic) and redilatation with a 24/20 mm ultrahigh pressure balloon inflated up to max. Twenty-two bar (Atlas gold, Bard) final result w/o residual gradient (E/F). RVOT: right ventricular outflow.

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Figure 3:Macroscopic view of the partially thickened valve cusps (A patient 1; B patient 4). Histology (Richardson blue stain) demonstrates significant thickening and retraction of valve cusps (C patient 1; D patient 4) and significant pseudointima proliferation containing fibromuscular cells embedded in abundant extracellular matrix without accumulation of inflammatory cells (E patient 1; F patient 4).

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Figure 4: A 29-mm No-React® Injectable BioPulmonicTM valve 7 years after implantation (Patient 1): valved stent tilted with horizontal orientation, not aligned to the right ventricular outflow axis and with consecutive sub- valvular narrowing; stent-struts deeply anchored when attached to the wall; moderate degree of neointimal proliferation (A/B). Test-ballooning (semi-compliant Cristal balloon (Balt, Montmorency, France) 25/50 mm) and simultaneously performed angiography via the long-sheath unmasked considerable paravalvular leakage on the left-anterior margin (C/D). We also noted close proximity of the caudal struts to the LAD artery (not shown) and decided against percutaneous re-revalvulation in this setting. LAD: left-anterior descending.

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Figure 5: A 29-mm No-React® Injectable BioPulmonicTM valve 7 years after implantation (Patient 4): obstruction at the level of the valve due to moderate tilt of the stent and neointimal proliferation resulting in an inner lumen of only 16.5–18 mm (A/B). Coronary angiography indicated the caudal strut-jags immediately next to the LAD (C/D). We refrained from percutaneous re-revalvulation due to the potential risk to the LAD after changing RVOT geometry via additional stenting. LAD: left anterior descending; RVOT: right ventricular outflow.

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Figure 6: A 23-mm No-React® Injectable BioPulmonicTM valve 5 years after implantation (Patient 5): dynamic sub-pulmonary narrowing (A), paravalvular flow and neointimal proliferation unmasked by test-ballooning with a 20/45 mm CBV (Balt), which occluded the valved stent completely at low inflation pressures correlating to an inner lumen around 17 mm (B). Deployment of a custom-made, 55 mm, covered 10-zig CP stent premounted on a 24/60 mm Balloon-in-Balloon system (NuMed) (C). After implantation of a second prestent (AS XXL 43 mm, Andramed), high-pressure ballooning up to 22 bar with a 24/20 mm Atlas balloon (Bard) and PPVI (Melody TPV, Medtronic), redilatation was performed with a 30/60 mm CBV balloon (D). Reasonable final result without residual paravalvular flow on angiography (E/F).

Take home points:

  1. The No-React® Injectible valve was introduced as a modern surgical valve replacement for minimally invasive and even off-pump procedures.
  2. The potential advantages were ease of implantation, favourable tissue constitution and a perfect landing zone for a percutaneous valve-in-valve.
  3. The long term results were disappointing.
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Fetal Cardiology Featured Articles of November 2015

1. Intrauterine Fetal Demise after Prenatal Diagnosis of Congenital Heart Disease: Assessment of Risk.

Divanovic A, Bowers K, Michelfelder E, Jaekle R, Newman T, Marcotte M, Habli M, Cnota JF.

Prenat Diagn. 2015 Nov 30. doi: 10.1002/pd.4755. [Epub ahead of print]

PMID: 26618782

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Select item 26616137

Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch: This study from Cincinnati was designed to determine the rate and risk factors associated of intrauterine fetal demise (IUFD) among infants with congenital heart disease (CHD).  In this study, the overall rate of IUFD was 4.7% (22/467).  Severe valve regurgitation, extracardiac abnormalities, and genetic abnormalities were found to be risk factors for increased IUFD in fetuses with CHD. A fetus with severe valve regurgitation was found to have a 7 fold increased risk of IUFD (OR 7.2, 95% CI 2.6-20.2) followed by a fetus with an extracardiac abnormality which had a 3 fold increased risk of IUFD (OR 3.4, 95% CI 1.4-8.3).   Researchers found a nearly 8 fold increase for IUFD if more than one risk factor (i.e. severe valve regurgitation, extracardiac abnormalities, or genetic abnormalities) were present (OR 7.8, 95% CI 2.3-26.6).  In comparison, the incidence of IUFD in fetuses with CHD with no risk factors was 1.2%.

Take Home Points: 

  1. The incidence of intrauterine fetal demise is low with CHD. The risk of IUFD increases with the presence of severe valve regurgitation, extracardiac abnormalities and/or genetic abnormalities.
  2. Severe valve regurgitation carried the highest risk of IUFD in fetuses with CHD.


2. Severity of Fetal Brain Abnormalities in Congenital Heart Disease in Relation to the Main Expected Pattern of in utero Brain Blood Supply.

Masoller N, Sanz-Cortés M, Crispi F, Gómez O, Bennasar M, Egaña-Ugrinovic G, Bargalló N, Martínez JM, Gratacós E.

Fetal Diagn Ther. 2015 Nov 28. [Epub ahead of print]

PMID: 26613580

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Select item 26612143

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch: This study from Barcelona, Spain supplements the growing number of studies demonstrating brain abnormalities in fetuses with congenital heart disease (CHD). In this study, 58 fetuses with CHD and 58 controls had a Doppler ultrasound and fetal MRI at 36-38 weeks gestation.  Fetuses with CHD were divided into 2 groups.  Group A were those fetuses with expected severe reduction in oxygenated brain blood supply (i.e. severe left outflow tract obstruction and transposition of the great arteries).  Group B were fetuses with CHD that would have near normal oxygenated brain blood supply.  On Doppler ultrasound the fetuses with CHD had significantly smaller mean biparietal diameters, smaller head circumference Z-scores, lower middle cerebral artery pulsatile index and lower cerebroplacental ratios compared to controls.  On fetal brain MRI, fetuses with CHD had significantly smaller total brain volume (TBrV), smaller total intracranial volume (TICV) and smaller opercular volume compared to controls.  There was also delayed cortical gyrification with decreased depths of fissures within the brain compared to controls.  There was significant linear tendency across the three subgroups with CHD group A fetuses showing the most pronounced differences.  See Figure.

Take Home Points: 

  1. This study demonstrates there are significant differences in the fetal brain at near term gestation in fetuses with CHD.
  2. Fetuses with expected severe reduction in oxygenated brain blood supply had the most significant differences.

fetal 2.1


3. Risk-Stratified Postnatal Care of Newborns with Congenital Heart Disease Determined by Fetal Echocardiography.

Donofrio MT, Skurow-Todd K, Berger JT, McCarter R, Fulgium A, Krishnan A, Sable CA.

J Am Soc Echocardiogr. 2015 Nov;28(11):1339-49. doi: 10.1016/j.echo.2015.07.005. Epub 2015 Aug 20.

PMID: 26298099

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Select item 25325374

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch:  Children’s National Medical Center (Washington, District of Columbia) has developed a level of care (LOC) protocol in which all fetuses diagnosed with congenital heart disease (CHD) are assigned a LOC by the fetal cardiologist and recommendations are created for postnatal management.  The LOC protocol is included in Table 1.  The purpose of this study was to assess the accuracy of fetal echocardiography to correctly diagnose CHD and predict postnatal care.  The LOC assigned by fetal echocardiography correctly predicted postnatal care in 427/463 fetuses (92%).  Sensitivity and positive predictive valves (PPV) for each LOC are as listed.

LOC 1 – sensitivity 90%, postnatal PPV 99%

LOC 2, sensitivity 97%, postnatal PPV 87%

LOC 3 and 4, sensitivity 83%, postnatal PPV of 87%

Researchers note that in their experience, postnatal compromise can be reliably predicted except in fetuses with d-transposition given the difficulty in predicting atrial and ductal level shunting.

Take Home Points: 

  1. The fetal echocardiogram and fetal cardiologist can usually predict the postnatal level of care of fetuses with CHD except in those fetuses with d-transposition.
  2. The level of care protocol developed at Children’s National Medical Center (Washington, District of Columbia) included in Table 1 is a validated tool to predict postnatal level of care in fetuses with CHD.

fetal 3.1


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