ACHD Featured Articles of March 2016

1. Hepatic stiffness in the bidirectional cavopulmonary circulation: The Liver Adult-Pediatric-Congenital-Heart-Disease Dysfunction Study group.

Kutty SS, Zhang M, Danford DA, Hasan R, Duncan KF, Kugler JD, Quiros-Tejeira RE, Kutty S.

J Thorac Cardiovasc Surg. 2016 Mar;151(3):678-84. doi: 10.1016/j.jtcvs.2015.09.079. Epub 2015 Sep 28.

PMID: 26515874

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Select item 26481279

Take Home Points:

  1. Hepatic stiffness is increased in very young children with single ventricle physiology after BCPC and before Fontan, and is mildly elevated compared to healthy age- and gender-matched controls.
  2. Hepatic stiffness measurement using shear wave elastography is feasible in small children and could be used to monitor disease progression.
  3. In a modern cohort of Fontan patients, who are now teenagers, hepatic stiffness is significantly higher than in young children awaiting Fontan palliation.

Kay_William_MD.16.CVaCommentary from W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch: Much has been written about liver disease in single ventricle patients after the Fontan.  Very little to date has been published regarding hepatic changes at earlier stages.  In this paper from the University of Nebraska, patients were evaluated for liver stiffness as infants at the bidirectional cavopulmonary connection (BCPC) phase, and compared to age-matched controls, as well as to patients who underwent Fontan palliation (n=20 BCPC patients, 20 controls, data compared to a historic cohort after Fontan procedure).

Patient were an average of 18 months status post BCPC surgery, and 90% had normal systolic function by echo, and 90% had mild or less AV valve regurgitation.  Half of patients had HLHS and the other half had a single left ventricle.  The investigators measured hepatic stiffness using shear wave elastography (SWE) and correlated this measurement with other parameters, such as portal vein flow and resistance of celiac and superior mesenteric arteries.  The hepatic stiffness in BCPC was significantly higher than in control subjects (7.2 vs 5.7 kPa, p = .039).  Patients after BCPC also had significantly higher celiac artery resistive index, pulsatility index, and systolic-diastolic flow ratio.  The portal vein flow was reduced in BCPC patients, with higher resistive index and higher pulsatility index than in controls.

A significant correlation was found between hepatic stiffness with right atrial pressure obtained at catheterization (p = .002).  However, patients at BCPC stage still had lower stiffness than the historical cohort of patients who had already undergone the Fontan procedure (7.2 vs 15.6 kPa, P < .001).  The historical cohort of Fontan data was published in 2014, by the same investigators, and was based on 41 patients, averaging 13.8 years in age, at an average of 11 ± 6 years after Fontan completion.

This paper is the first paper to report on findings of hepatic stiffness based off shear wave elastography in children with single ventricles after the BCPC and prior to Fontan completion.  It is remarkable that out of only 22 patients in their entire program awaiting Fontan completion, 20 were enrolled in the study.  More robust data will thus require multicenter investigation.  It is uncertain if the elastography measures intrinsic liver stiffness, or if (potentially reversible) elevations of central venous pressure also raises the level of stiffness assessed in kPa.  There are numerous other causes for liver damage, other than congestion, in single ventricle patients, including hypoxia, CHF, shock, palliative shunting, and the effects of cardiopulmonary bypass.  However, this study is a nice starting point, and this technique could feasibly be used to a broader number of patients at more centers to obtain serial measurements.

A key limitation to this study is that hepatic stiffness was not assessed prior to BCPC, so BCPC itself cannot be assumed to be a cause of hepatic stiffness based on the data obtained.  Another limitation is that there was no correlation with liver biopsy, given ethical limitations in doing liver biopsies on young children without obvious clinical indication.

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FIGURE 1. Shear wave elastogram and vascular Doppler indices are shown. Elastogram maps show hepatic stiffness measurements by SWE in (A) a subject who had BCPC, and (B) a healthy subject. Hepatic ultrasound Doppler measurements are shown for (C) the main portal vein; (D) the celiac artery; and (E) the superior mesenteric artery. Min, Minimum; Max, maximum; Diam, diameter; SWE, shear wave elastography; BCPC, bidirectional cavopulmonary connection; PSV, peak systolic velocity; EDV, end-diastolic velocity; RI, resistive index; PI, pulsatility index; AT, acceleration time; TAPV, time average peak velocity; TAMV, time average mean velocity; AI, acceleration index; PG, peak gradient; VTI, velocity-time integral; HR, heart rate; VFlow, flow volume.



2. Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow.

Ono M, Vogt M, Cleuziou J, Kasnar-Samprec J, Burri M, Strbad M, Hager A, Schreiber C, Hörer J, Lange R.

Ann Thorac Surg. 2016 Mar;101(3):1012-9. doi: 10.1016/j.athoracsur.2015.09.085. Epub 2015 Dec 8.

PMID: 26680312

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Select item 26830903

Take Home Points:

  1. Patients after the Björk modification of the Fontan procedure have larger right ventricles and better exercise capacity if they have pulsatile rather than nonpulsatile pulmonary blood flow. Exercise capacity of these patients was similar to that of patients who have had more modern TCPC Fontan operations.
  2. At rest, the only difference in hemodynamics between the groups was a higher peak systolic pulmonary artery blood pressure in the group with pulsatile pulmonary blood flow, but there was no difference in mean RA pressure, mean PA pressure, PVR, or end-diastolic pressure. Given the improved exercise capacity in Group P over Group N, it is likely that there would be a different invasive hemodynamics during exercise.
  3. No significant predictor was found in preoperative data between Group P and Group N that could have predicted preoperatively which patients would develop pulsatile pulmonary blood flow. However, there was a higher transpulmonic gradient in 24/30 in Group N who had never had pulsatile flow versus the combination of the Group P patients and the 6/30 in Group N who initially had but lost pulsatile blood flow.
  4. If reliable factors that can predict pulsatile systolic pulmonary blood flow could be identified, the Björk modification could potentially be offered in a select subset of patients with tricuspid atresia.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  The Björk modification of the Fontan, performed only for the diagnosis of tricuspid atresia or double-inlet left ventricle, is an abandoned procedure in which a surgical connection (see Figure below) is made to connect the right atrium to the right ventricle.   This procedure was eventually abandoned in favor of the TCPC (extracardiac or lateral tunnel Fontan) due to concerns of stenosis and regurgitation over the atrioventricular connection, as well as due to arrhythmias and unfavorable hemodynamics.

The rationale behind the Björk modification is to include the RV as a potential pumping chamber to augment forward flow to the pulmonary circulation.  Prior studies had shown no advantage to this versus the classic atriopulmonary connection, but some reports had shown that the RV in some cases grew significantly.  A hypothesis was thus made that patients with pulsatile systolic pulmonary blood flow would have larger RVs and thus would have better outcomes.

This study, from Munich, Germany, tested this hypothesis by evaluating  long term outcomes of 41 patients after the Björk modification, and compared complications of those with pulsatile pulmonary flow (Group P, n = 11) with those without no pulsatility of pulmonary flow (Group N, n = 30).  Parameters evaluated included hemodynamics, right ventricular size, exercise capacity, and clinical outcomes.  Mean follow-up time was over 25 years in both groups.  Prior to 1995, 173 patients at Munich had undergone classic Fontan (n=106 atriopulmonary connection, n=67 Björk modification).  Mean age of Björk modification was 6.2 ± 5.0 years.  Out of 67 who initially underwent the Björk modification, there were 2 early and 13 late deaths (22%).  A few others were lost to follow-up, and 41/52 survivors have been actively followed at the center.  Patients underwent cardiopulmonary exercise testing, with measurement of peak oxygen uptake (VO2).   Selected patients underwent cardiac catheterization and cardiac MRI if needed for clinical purposes.

Absence of pulsatile systolic pulmonary artery blood flow was defined as pulseless continuous systolic-diastolic flow, respiratory-dependent pulmonary flow, or flow with minimal acceleration in the end-diastolic phase (Figure 1, below).

Late atrial tachyarrhythmia occurred in 85% of the cohort.  Interestingly, despite the need for an RV incision with this procedure, no ventricular arrhythmia was observed.  Patients in Group P required fewer catheter ablation procedures for atrial arrhythmias than those in Group N (9% versus 50%, p = 0.03).  In the subset who underwent cardiac MRI, measurements of RV volumes were significantly larger in Group P than in Group N.  By MRI, there was no difference in LV volumes between groups.   Although preoperative and intraoperative records indicated significant hypoplasia of all RVs prior to surgery, at the last follow-up, 10/11 patients in Group P had reached normal (or greater than normal) RV size, whereas 20/30 in Group N still had a hypoplastic RV.  Of note, six patients in Group N had initially had pulsatility of pulmonary blood flow, but eventually lost it, whereas all patients in Group P had pulsatile blood flow on every echocardiogram during the study period.  An interesting finding was that 5/6 in Group N who initially had pulmonary pulsatility had developed normal RV size, even though they lost pulsatility.  Pulsatility was lost anywhere from 12 years to 32 years after Björk modification.

Exercise capacity was higher in Group P than in Group N.  Peak VO2 was 25.0 ± 7.3 versus 19.6 ± 6.0 mL/kg/min, respectively (p = 0.03), and VO2 at anaerobic threshold was 14.6 ± 4.7 versus 11.5 ± 3.6 ml/kg/min, respectively.

It should be noted that the echocardiograms used for inclusion in the study were the last echocardiogram performed in clinic for routine purposes, or the last echocardiogram prior to conversion to a total caval pulmonary connection (TCPC).  A variety of materials were used to form the RA-RV connection, including pericardium, Dacron, GoreTex, and homograft.

The authors concluded by developing a few hypotheses as to why patients in Group P had larger RVs and better exercise capacity.  Possible reasons presented were  1) pulsatile pulmonary blood flow may result in improved right ventricular compliance and thus promote a larger RV which can generate more stroke volume, 2) patients with pulsatile pulmonary blood flow might have better RV/LV interactions, and 3) pulsatile flow might increase more with exercise than nonpulsatile flow.  Unfortunately, no significant difference could be identified in the preoperative variables between Group P and Group N which could identify which patients would likely end up with pulsatile versus nonpulsatile pulmonary blood flow at late follow-up.  The authors did mention at the end of the paper that when combining Group P along with the 6 patients in Group N who initially had pulsatile flow, but lost it later, and comparing them to the other 24 patients in Group N, that there was a lower transpulmonary gradient, so perhaps a low PVR may have been the key difference that led to at least early pulsatility of pulmonary blood flow.

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FIGURE – schematic of Bjork modification showing surgical RA-RV connection

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Fig 1. Parasternal short-axis echocardiographic view of the main pulmonary artery after Fontan-Björk procedure. (A) Pulsatile Doppler systolic flow in the main pulmonary artery of a patient in group P. (B) Nonpulsatile systolic diastolic flow in the main pulmonary artery of a patient in group N.



3. Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience.

Ono M, Kasnar-Samprec J, Hager A, Cleuziou J, Burri M, Langenbach C, Callegari A, Strbad M, Vogt M, Hörer J, Schreiber C, Lange R.

Eur J Cardiothorac Surg. 2016 Mar 23. pii: ezw091. [Epub ahead of print]

PMID: 27009106

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Select item 27009890

Take Home Points:

  1. TCPC in Munich had a 98.2% 30-day survival and 93.2% 15-year survival.
  2. In Munich, partial cavopulmonary connection was performed at 3 months, and TCPC at 18-24 months, with extracardiac conduits now being procedure of choice. Fenestrations are very rarely done at this center (only 5 patients have undergone primary fenestration since 2001).
  3. When compared with classic Fontan, TCPC has dramatically decreased the incidence of tachyarrhythmia, need for reoperation, and thromboembolic complications.
  4. In this study, patients with extracardiac Fontan had significantly higher exercise capacity than those with lateral tunnel Fontan (VO20% predicted versus 59.9% predicted, respectively). Reasons for this are unclear.
  5. Freedom from late tachyarrhythmia was 91% at 15 years post TCPC. This is similar to other studies of large TCPC cohorts.
  6. PLE and plastic bronchitis were extremely rare in this cohort of patients. The only multivariable predictor of PLE in this study was an elevated PA pressure.
  7. Thromboembolic complications were exceedingly rare in this study, but this center has an institutional policy that all patients are treated with vitamin K antagonists with a target INR of 2.0-3.0.

Commentary  from W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  This study from Munich, Germany sought to evaluate long-term outcomes after total cavopulmonary connection-type Fontan procedures in single ventricle patients.  Over a 21 year period, 434 patients underwent TCPC at their center (n=50 lateral tunnels and n=384 extracardiac conduits).  Clinical endpoints, exercise capacity, and liver evaluations were reviewed retrospectively.  At the Munich center, the lateral tunnel was first utilized in 1994, the extracardiac conduit was first introduced in 1999, and since 2002, the extracardiac conduit has been their procedure of choice.  Patients who initially had a classic Fontan and were converted to TCPC (n = 33) were excluded from analysis in this study.  Early mortality was rare (8 deaths < 30 days postop, 3 in lateral tunnel, 5 in extracardiac), with overall 30 day survival of 98.2%.  Of the 426 early survivors, 10 patients were lost to follow-up after hospital discharge.  Mean follow-up period was 6.6 ± 5.4 years in all patients in the study.

Median age at TCPC was 2.3 years, with a median weight of 12.0 kg.  Most patients had a partial cavopulmonary connection prior to Fontan (88.7%).  Only one patient underwent Fontan take-down over the study period.  In Munich, the TCPC is performed at an age of 18-24 months old, and uses an 18 mm conduit in the majority of cases.  In those with prior Kawashima operation, TCPC is performed at a median age of 36 months.  Fenestration was placed when clinically indicated per the operators, with a total of 35 (8%) fenestrated procedures (48% in lateral tunnel, 6% in extracardiac conduit, p < 0.001).

Overall Kaplan-Meier 15 year survival was 92.3%.

On multivariate analysis, predictors of prolonged hospital stay or other significant clinical endpoints included a high transpulmonic gradient, hypoplastic left heart syndrome, a higher number of pre-Fontan palliations, significant AV valve regurgitation, prior Norwood procedure, dextrocardia, high mean aortic pressure, and high pulmonary artery pressure.  Postoperative complications including long hospital stay, long duration of chest tube drainage, and prolonged ICU stay were strongly correlated with late mortality, reoperations, and interventions.   See Table 6 from paper for full details.

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4. Cause of death in adults with congenital heart disease – An analysis of the German National Register for Congenital Heart Defects.

Engelings CC, Helm PC, Abdul-Khaliq H, Asfour B, Bauer UM, Baumgartner H, Kececioglu D, Körten MA, Diller GP, Tutarel O.

Int J Cardiol. 2016 Mar 2;211:31-36. doi: 10.1016/j.ijcard.2016.02.133. [Epub ahead of print]

PMID: 26970963

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Select item 26803381

Take Home Points:

  1. Community based registries can complement data obtained from tertiary care clinical studies. Although data is not always complete or verifiable, using a community based registry can avoid selection bias inherent in studies at large university centers.
  2. The majority of patients with CHD who die in adulthood die of CHD-related complications, with progressive CHF being the most common cause, followed by sudden cardiac death.
  3. There is an increasing complexity of CHD surviving to adulthood, manifested by an increasing number of patients with severe CHD dying in adulthood.
  4. Higher severity of CHD correlates with a higher likelihood of having a CHD-related death.
  5. Those that die of CHD-related deaths are still dying at fairly young ages in their 4th decade and there is significant room to improve this.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  The German National Register for CHD is voluntary for patients or parents of infants diagnosed with CHD.  This study evaluated causes of death amongst the ACHD population from 2001-2015, and adds to valuable data from prior studies in Finland, Canada, and the United Kingdom.  Unlike most prior studies, this is a population-based study, rather than a study from a single tertiary care ACHD center, thus there should be less selection bias.

A total of 2596 patients were analyzed, of which 9.2% died over a median follow-up of 3.67 years.  Patients were divided into 5 groups based on prior surgical history, including 1) no surgery, 2) corrective surgery (e.g. tetralogy of Fallot complete repair), 3) palliative surgery (i.e. transposition with atrial switch), 4) Fontan procedure, and 5) transplant.  The patients were further categorized into complexities of CHD based on the Bethesda classification, and the mode of death was classified as CHD-related or non-CHD related.  CHD-related deaths included progressive heart failure, perioperative death, sudden death, infection (endocarditis), or complications after heart or lung transplantation (infection or acute rejection).

Cause of death was determined either through medical records or by contacting the personal family physician of the deceased.  The cause of death was CHD-related in 71% of the study patients, non-CHD related in 18%, and unknown in 10% of patients.  Non-CHD related deaths were the leading cause of death in patients with simple CHD such as septal defects and coarctation.  Sudden cardiac death was the leading cause of death in Eisenmenger Syndrome, Tetralogy of Fallot, and Marfan syndrome.  There were 5 sudden deaths in transposition of the great arteries (the study did not specify if these were atrial switch or arterial switch patients).

Due to significant increases in utilization of implantable cardioverter-defibrillator around 2008 in Europe, the study period was further subdivided from 2001-2008 and 2009-2015 to evaluate differences in mortality and comorbidities between those two time periods.

Not surprisingly, patients who died during the study were more likely to be NYHA class III or IV than patients who did not die.  Patients who died of CHD-related causes were more likely to have more complex CHD.  The mean age of death for the whole study period was 39.8 ± 17.8 years, but the mean age for CHD-related death was 34.5 ± 13.1 years versus 57.6 ± 20.5 years.

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Fig. 2. Causes of death compared between time period I (January 2001–December 2008) vs. II (January 2009–January 2015).

When comparing the early era (2001-2008) versus the later era (2009-2015), mean age of death was higher in the earlier time period 41.5 ± 18.9 years whereas in the more recent period mean age of death was lower at 38.9 ± 17.2 years.  This was likely driven by a lower number of children dying and thus dying as adults rather than as children, thus driving down the average age of death, bearing in mind that the vast majority of patients in the study are still alive.  Out of the 160 patients who died in the more recent time period, 63% had severe CHD, and 25% had moderate CHD, whereas out of the 79 patients who died in the earlier time period, 18% had simple CHD, 29% had moderate CHD, and 53% had severe CHD.

An alarming finding in this study is that perioperative mortality was the cause of death in 16.3% of patients who died, with the majority of this occurring with reoperations.

The percentage of patients dying of sudden cardiac death decreased from 29% in the earlier time period to 20% in the later time period; this may likely be due to an increased utilization of ICDs over the course of the study period.

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Fig. 3. Causes of death grouped for individual congenital heart defects. VSD: ventricular septal defect; UVH: univentricular heart; TOF: Tetralogy of Fallot; TGA: transposition of the great arteries’ Others: cor triatriatum sinistrum, mitral valve anomaly, pulmonary artery anomaly, double outlet right ventricle (transposition-type), truncus arteriosus communis, aortopulmonary window, Shone complex, abnormal origin of the coronary artery from the pulmonary artery, pulmonary stenosis, mitral valve prolapse; CoA: coarctation of the aorta; ccTGA: congenitally corrected TGA; AVSD: atrioventricular septal defect; ASD: atrial septal defect; AS: aortic stenosis.



5. Rehospitalization Is a Major Determinant of Inpatient Care Costs in Adult Congenital Heart Disease.

Cedars AM, Burns S, Novak EL, Amin AP.

J Am Coll Cardiol. 2016 Mar 15;67(10):1254-5. doi: 10.1016/j.jacc.2015.12.043. No abstract available.

PMID: 26965547

Similar articles

Select item 26993217

Take Home Points:

  1. Readmissions and surgical operations in ACHD are common and expensive
  2. If certain readmissions could be prevented, significant money could be saved in annual hospital charges for ACHD patients
  3. This issue requires further study, but studies are limited due to the quality of data in administrative databases.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch: This study analyzed data from 6 states in the United States with statewide inpatient databases.  The individual states were selected because their databases allowed for longitudinal follow-up of the same patient over multiple admissions via unique identifiers.

Admissions for ACHD-related reasons were identified by screening ICD-9 codes with further correction to adjudicate diagnosis via the Broberg algorithm.  Patients were excluded if their index admission was in the first 12 months or last 12 months of the study period.

Data was analyzed to identify patients above and below median costs over a 12-month period.  This data was further analyzed against published data as well as the investigators’ clinical experience in order to determine potential variables that may correlate with inpatient care costs.  The primary outcome of the study was total financial burden over a 12-month period.

There were 155,297 index admissions with 619,720 readmissions.  A total of 112,113 readmissions occurred within 1 year of the index admission.  After adjudication of cases that were not truly ACHD admissions, and after excluding index admissions in the 1st or last 12 months, and after excluding admissions for which there was no cost data available, the final sample size was 68,314 admissions.

The average age of ACHD patients who were admitted to the hospital was 57.0 ± 18.6 years, with 51% women.  A large percentage (40%) of these admissions were for surgical operations.  Readmissions were expensive and accounted for an average of $23,993 over a 12 month period.  The cost was $16,822 greater for patients who required surgery.  Although most surgical procedures cannot be avoided, the authors proposed that greater efficiency that might prevent readmissions could save significant costs.

The study was limited due to its use of administrative data which is not verifiable for completeness and accuracy.  Patients who left the 6 states would have been lost to follow-up in this study, and death is not accounted for in these inpatient databases, thus that is a potential source for error.



6. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension.

D’Alto M, Dimopoulos K, Budts W, Diller GP, Di Salvo G, Dellegrottaglie S, Festa P, Scognamiglio G, Rea G, Ait Ali L, Li W, Gatzoulis MA. Heart. 2016 Mar 24. pii: heartjnl-2015-308903. doi: 10.1136/heartjnl-2015-308903. [Epub ahead of print] Review. PMID: 27013702 Similar articles Select item 27018378

Take Home Points:

  1. A simple chest x-ray may be very useful in the evaluation of PAH-CHD patients.
  2. The Moceri composite risk score may be a valuable tool for the assessment of PAH-CHD patients with echo.
  3. Right heart catheterization remains the gold standard for diagnosis and individual management of patients with PAH-CHD.

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: A multimodal approach to the evaluation of complex congenital heart disease and comorbidities is becoming the gold standard of adult congenital heart disease management in the digital age. These European authors describe their approach specifically to evaluating pulmonary arterial hypertension in patients with congenital heart disease. This article discusses the use of standard chest radiography, echo, Cardiac CT, and Cardiac MRI and their risks, benefits, and applications. CXR is simple, inexpensive, and has low levels of radiation. The information of the cardiothoracic ratio is reproducible, and has diagnostic and prognostic implications. Changes in cardiac size can be followed longitudinally as a response to therapy in locations where access to more advanced imaging may be limited. Echo is valuable in assessing prognosis, and response to therapy. Moceri et al suggest that a composite risk score based on TAPSE <15 mm, RV effective systolic to diastolic duration ratio >1.5, right atrial area >2.5cm2, and right atrial to left atrial area ratio >1.5 is highly predictive of clinical outcome. CMR allows for monitoring of structural and functional changes associated with CHD-PAH, primarily affecting the RV and the pulmonary circulation.  CMR is a low risk and radiation-free diagnostic tool, but is limited by availability to tertiary and quaternary care centers, as well as limited by adequately trained technicians, and faculty. There are some limitations related to artifact of implanted pacemaking systems in the pulmonary system which limits interpretability of flows. Cardiac CT can be used when CMR is not an option although it requires increased radiation exposure as well as iodinated contrast exposure. However, CT is considered the modality of choice for assessment of the great vessels including proximal and distal pulmonary artery anatomy, and presence of thrombus.  Cardiac catheterization remains the gold standard for diagnosis and management of PAH-CHD as no other modality is able to completely assess step-wise pressures and outputs, as well as allow for initiation of therapy during the procedure.

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7. Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation.

Broomall E, McBride ME, Deal BJ, Ducharme-Crevier L, Shaw A, Mazwi M, Backer CL, Mongé MC, Costello J, Marino BS, DeFreitas A, Wainwright MS. Ann Thorac Surg. 2016 Mar 23. pii: S0003-4975(16)00002-3. doi: 10.1016/j.athoracsur.2016.01.001. [Epub ahead of print]

PMID: 27016841 Similar articles Select item 27005935

Take Home Points:

  1. Vertebrobasilar stroke carries poor outcomes in Fontan patients undergoing revision or heart transplantation.
  2. Pre-operative identification of altered posterior cerebral circulation allows for customizing the management of perioperative (Fontan revision/transplant) risk.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch:: This article highlights the need for multidisciplinary care and evaluation of Fontan patients prior to undergoing Fontan revision, or heart transplant. Cerebrovascular anatomy may be altered with the use, occlusion, or ligation of subclavian arteries in staged pre-Fontan palliation. This changes flow to vertebral arteries and may compromise flow in patients with functionally univentricular hearts. Although this is a small series of patients, the Chicago group made a fundamental shift in their evaluation and care of patients that would lend itself to wider study, and should at least suggest the possibility that further investigation by neuroradiology, and neurocritical care is recommended prior to undergoing heart transplant or Fontan revision.  The strategic changes included higher perioperative blood pressures, advanced neuroimaging, and individual tailoring of therapy. Cerebral perfusion was maintained at >50 mmHg in the setting of normal ICP. This small case series documents abnormal posterior circulation anatomy.  The demonstration of atrophic vertebral arteries allows for customization of peri-operative management strategy. Posterior circulation stroke has very poor outcomes. Posterior circulation compromise has not previously been identified as a long-term risk in univentricular heart palliation or in Fontan failure.



9. Bodily isomerism is an independent risk factor for pulmonary hypertension in adults with congenital heart disease.

Loomba RS, Aggarwal S, Arora RR, Anderson R. Ther Adv Respir Dis. 2016 Mar 4. pii: 1753465816636810. [Epub ahead of print] PMID: 26944361 Similar articles Select item 27032711

Take Home Points:

  1. Isomerism poses a 79% increase over baseline for risk of pulmonary arterial hypertension.
  2. Fetal counseling as well as routine education and life-long screening regarding development of PAH-CHD is recommended.
  3. The risk of PAH-CHD with isomerism is still only 5.6%.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: Heterotaxy and isomerism have multiple associated comorbidities that are not associated with the cardiovascular system. This article highlights a long-term comorbidity that may be overlooked in the clinical follow up and evaluation of these already complex adult congenital patients. Patients with heterotaxy spectrum may have a two-ventricle repair with arterio-ventricular concordance, or they may have a single, morphologic right ventricle with interrupted IVC, and hemiazygous continuation, or anything in between. This article highlights a group of patients who were extracted from the Nationwide Inpatient Sample with 861 patients with isomerism, and 5.6% of those patients met criteria for PAH-CHD vs. only 2.6% without.  The predictive risk factors in this population are increasing age, increasing BMI, and history of anomalous pulmonary venous connection. The authors suggest that patients with isomerism should be counseled regarding PAH-CHD at the time of diagnosis, and should be included in prenatal counseling where applicable.



10. BMPR2 mutation is a potential predisposing genetic risk factor for congenital heart disease associated pulmonary vascular disease.

Liu D, Liu QQ, Guan LH, Jiang X, Zhou DX, Beghetti M, Qu JM, Jing ZC. Int J Cardiol. 2016 Mar 7;211:132-136. doi: 10.1016/j.ijcard.2016.02.150. [Epub ahead of print] PMID: 27002414 Similar articles Select item 26956707

Take Home Points:

  1. BMPR-2 missense mutations are common (9.9%) in female patients with repaired congenital heart disease.
  2. BMPR-2 mutations are strongly associated with progressive pulmonary arterial hypertension.
  3. BMPR2 mutations should be sought in CHD patients who develop PAH.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This study evaluated 294 patients with PAH-CHD in China. The patients were diagnosed with PAH-CHD after an initial screening echocardiogram was suggestive of PAH-CHD. The PVR was required to be >3 Woods Units. BMPR2 mutation carries a well-known correlation with familial pulmonary arterial hypertension. It is most commonly found in female carriers, and is typically associated with identification of iPAH in juveniles.  A significantly higher BMPR2 mutation rate was found in repaired CHD.  Missense mutation of BMPR2 is the dominant mutation type.  Female repaired CHD patients are most likely to have the BMPR2 mutation detected. In patients with repaired PAH-CHD, 7.5% were BMPR2 mutation positive compared to 1.2% of patients without PAH. In female patients the mutation rate reaches 9.9%, this approaches the mutation rate in Chinese iPAH. Genetic predisposing factors may be important elements in the process of development of PAH in CHD patients. Female, repaired patients are more likely to be detected with genetic mutations.

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