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.
Select item 26474467
Comment 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.
Blok IM, van Riel AC, Mulder BJ, Bouma BJ.
Expert Rev Cardiovasc Ther. 2015 Oct 16:1-16. [Epub ahead of print]
Select item 26474570
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.
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.
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.
Butts RJ, Chowdhury SM, Baker GH, Bandisode V, Savage AJ, Atz AM.
Pediatr Cardiol. 2015 Sep 26. [Epub ahead of print]
Select item 26408307
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]
Select item 26482350
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.
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.
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.
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.
Select item 26117295
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.
Lanz J, Brophy JM, Therrien J, Kaouache M, Guo L, Marelli AJ.
Circulation. 2015 Nov 23. pii: CIRCULATIONAHA.115.011241. [Epub ahead of print]
Select item 26596791
Comment 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:
- 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.
- Stroke incidence was considerably higher than in the general population, especially at a younger age.
- 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.