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Is Grown Up Congenital Heart (GUCH) disease different in a developing country?

J Pak Med Assoc. 2016 10;66(Suppl 3)(10):S5-S7

Authors: Shahabuddin S, Hashmi S, Rakhshan SE, Khan JK, Sami SA, Amanullah M

In the current era grown up congenital heart disease (GUCH) patients undergoing surgical interventions are increasing. Most of the interventions in the developed countries are either complex or redo-operations in patients who had previously undergone repair, palliation or correction. However, in the developing countries most of the interventions are primary and corrective. This descriptive retrospective study comprised GUCH patients who underwent surgical intervention for congenital heart disease (CHD) at Aga Khan University Hospital, Karachi, from January 2006 to December 2015. A total of 195 patients had been treated surgically with a mean age of 31.0±13.5 years. Majority of the patients underwent surgical interventions for closure of atrial 109(55.3%) and ventricular 51(26.2%) septal defect. The most common complications were prolonged ventilation 16(8.1%). Overall mortality was 4(2.1%). GUCH in our practice is for primary procedure with simple diagnosis that should have been treated before reaching adulthood as is done in the developed countries.

PMID: 27895340 [PubMed - indexed for MEDLINE]

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Complex Congenital Heart Disease: Is Heart Transplantation an Option?

Transplant Proc. 2018 Mar;50(2):655-657

Authors: Esteve-Ruiz I, Grande-Trillo A, Rangel-Sousa D, Adsuar-Gómez A, Sobrino-Márquez M, Lage-Gallé E

INTRODUCTION: Within the heart transplant (HT) population, few patients have complex congenital heart disease (CHD) underlying disease. Our objective is to assess the complications and follow-up of patients with CHD transplanted in our center (1991-present).
MATERIAL AND METHODS: Retrospective analysis of patients older than 14 years old with CHD and HT. Clinical and surgical variables were analyzed.
RESULTS: Ten patients (age 25 ± 7 years old, 60% male) were analyzed, which represents 2.2% of the entire series of HT; 9 of the 10 patients were electively transplanted and 1 was under support with extracorporeal membrane oxygenation. Compared with the rest of the transplanted patients, patients with CHD had a lower median age (25 [25-36] vs 53 [15-69]; P < .009); more cardiac surgeries prior to HT (100% vs 14.4%; P < .001); a lower percentage of cardiovascular risk factors (CVRF; 0% vs 60%; P < .001). The left ventricle ejection fraction also showed statistically significant differences (33.5 [12-67] vs 20 [6-70]).
CONCLUSIONS: Our patients with CHD and HT are younger and have less CVRF and more cardiac surgeries, which highlights that it is a subgroup with clear clinical differences in its comorbidity and pretransplantation assessment. They also require longer extracorporeal circulation time, more hours of intubation, and more days in the intensive care unit. Primary graft failure is more common in patients with CHD. Therefore, survival at 1-month follow-up is lower than the rest of the series and equalizes after the year of follow-up. This long-term survival reaffirms the possibility of HT in CHD despite the fact that its postoperative period is more difficult.

PMID: 29579880 [PubMed - indexed for MEDLINE]

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Bridge to Transplantation with Long-term Mechanical Assist Devices in Adults with Transposition of the Great Arteries.

Artif Organs. 2018 Aug 21;:

Authors: Michel E, Orozco Hernandez E, Enter D, Monge M, Nakano J, Rich J, Anderson A, Backer C, McCarthy P, Pham D

BACKGROUND: Prior to the widespread adoption of the arterial switch operation, patients with transposition of the great arteries (TGA) commonly underwent atrial switch operation (Mustard or Senning). It is not uncommon for these patients to progress to end stage heart failure and increasingly, ventricular assist devices (VADs) are used to support these patients as a bridge to transplantation, though there is limited experience with this worldwide.
METHODS: A retrospective review of our institution's VAD database was undertaken and revealed seven adult patients with a history of TGA and subsequent systemic ventricular failure were implanted with a VAD. Four of whom received the VAD as a bridge to transplantation (BTT) at the time of implantation, 2 who were initially designated as destination therapy secondary to severe pulmonary hypertension, and 1 who was designated as destination therapy secondary to a high risk of life threatening non-compliance.
RESULTS: Seven patient cases who received a VAD for severe systemic ventricular failure were included in this study. The mean age of the patients was 40 years and the majority of patients were male (6/7, 85%). Five of the patients (71.4%) had previously undergone an atrial switch operation and all of these were Mustard procedures. Two of the seven patients (28.5%) had congenitally corrected transposition of the great arteries (CC-TGA). Two of the seven patients (28.5%) had supra-systemic pulmonary pressures before VAD implantation and were designated as Destination Therapy (DT). One of these patients was later designated as BTT as an improvement in his pulmonary vascular resistance was observed, and subsequently underwent heart transplantation. Because of anatomic considerations, four of the patients (57%) underwent redo-sternotomy with outflow cannula anastomosis to the ascending aorta, 1 patient underwent VAD implantation via a left subcostal incision with anastomosis of the outflow graft to the descending thoracic aorta, and 2 patients (28.5%) underwent VAD implantation via a left thoracotomy and anastomosis of the outflow cannula to the descending thoracic aorta. Six of the seven patients had a HeartWare® HVAD VAD implanted; one received a Thoratec ® Heartmate II VAD. Two patients underwent VAD explant and orthotopic heart transplant, 222 days and 444 days after VAD implant, respectively. One patient died on post-operative day 17 after complications from recurrent VAD thrombosis despite multiple pump exchanges. Four patients remain on VAD support, three of these patients are awaiting transplantation at last follow-up (mean days on support, 513 days).
CONCLUSIONS: Bridge to transplantation with a durable VAD is technically feasible and relatively safe in patients with TGA. Multiple redo sternotomies can be avoided with a left posterior thoracotomy approach and outflow graft anastomosis to the descending thoracic aorta after careful anatomic considerations. This article is protected by copyright. All rights reserved.

PMID: 30129258 [PubMed - as supplied by publisher]

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Does Tetralogy of Fallot affect brain aging? A proof-of-concept study.

PLoS One. 2018;13(8):e0202496

Authors: Codari M, Papini GDE, Melazzini L, Pluchinotta FR, Secchi F, Carminati M, Frigiola A, Chessa M, Sardanelli F

The impact of congenital heart disease on brain aging has not been extensively investigated. We evaluated cerebral microbleeds and white matter hyperintensities on brain magnetic resonance imaging in adult patients with tetralogy of Fallot (ToF). Ten ToF patients (6 women, 4 men; aged 21-58 years; New York Heart Association [NYHA] class 1-2) were prospectively enrolled and underwent a T1-weighted, a T2-weighted dark fluid, and a T2*-weighted scans. Ten age- and sex-matched controls were prospectively recruited and subjected to the same acquisition protocol. Cerebral microbleeds (CMBs) were manually counted while white matter hyperintensities (WMHs) were segmented using ITK-Snap. Wilcoxon signed-rank test, Spearman correlation, and Bland-Altman statistics were used. The median (interquartile range [IQR]) age was 45.0 (30.5-49.5) years in ToF patients and 46.0 (30.5-49.8) years in controls. The median (IQR) of the number of CMBs was 6.0 (4.0-7.8) in ToF patients and 0 (0.0-0.0) in controls (p = 0.002). The WMHs burden was 2,506 (1,557-2,900) mm3 for ToF patients and 2,212 (1,860-2,586) mm3 for controls (p = 0.160). Moreover, a positive significant correlation was found between the WMHs burden and the NYHA class (ρ = 0.80, p = 0.005). Inter-operator concordance rate for the presence/absence of CMBs was 90%; the reproducibility for the WMHs burden was 77%. In conclusion, we found more cerebral microbleeds and a higher WMHs burden in adult ToF patients than in controls. This preliminary comparison supports the hypothesis of an early brain aging in ToF patients. Larger studies are warranted.

PMID: 30130369 [PubMed - in process]

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Impact of maternal pulmonary insufficiency on fetal growth in pregnancy.

J Matern Fetal Neonatal Med. 2018 Aug 21;:1-151

Authors: Brun S, L'Ecuyer E, Dore A, Mongeon FP, Guedon AC, Leduc L

RATIONALE: It is known that fetal growth is usually proportional to left-sided cardiac output (CO) which parallels the right-sided CO and that congenital right-sided lesions are usually associated with better perinatal outcomes than left-sided lesions.
OBJECTIVE: Our objective was to document whether newborns from mothers with severe residual pulmonary valve insufficiency (PI) after surgical tetralogy of Fallot (TOF) or pulmonary stenosis (PS) correction have lower birth weight (BW) than newborns from mothers with absent, mild or moderate PI.
METHODS: This is a retrospective cohort study of women affected with repaired TOF and corrected PS with varied severity of residual PI. Exclusion criteria were: left ventricular dysfunction, left-sided valvular heart disease, other right-sided structural heart disease, chronic hypertension, substance addiction and incomplete follow-up. Pregnancies were divided into three groups: absent or mild PI, moderate PI and severe PI. A generalized linear model with normal dependent variable distribution was built as well as the parameter estimation made with Generalized Estimation Equations (GEE) in order to take into account repeated mother in data. Variables such as gestational age at birth, maternal age, smoking and body mass index were tested with bivariate analyses to assess their effect on BW. Only gestational age remained in the adjusted model.
RESULTS: A total of 45 patients were included (33 TOF and 12 PS) and 97 pregnancies were reported: 22 miscarriages (22.7%) (15 TOF, 7 PS) and 75 successful pregnancies (57 TOF, 18 PS). The patients were divided into three groups: 1) Absent or mild PI, 2) moderate PI and 3) severe PI groups which comprised respectively 29 (15 TOF, 4 PS), 20 (10 TOF, 1 PS) and 26 successful pregnancies (8 TOF, 7 PS). Using three levels of PI (absent or mild, moderate and severe), the unadjusted model showed a significant effect of level of PI on BW (p = 0.0118) as well as the adjusted model (p = 0.0263) with gestational age as covariate. The estimated mean newborn's BW was 3055.8 g in the severe PI group, 3151.0 g in the moderate PI group and 3376.4 g in the absent or mild group when adjusted for gestational age. Hence, we estimated that the mean newborn's BW is 321 g lower in the severe PI group compared to absent or mild PI group ((CI: 572.3; -68.9), p = 0.0087).
CONCLUSION: Pregnancy is usually well tolerated in repaired TOF and corrected PS. Severe PI either from repaired TOF or PS is at higher risk of lower newborn's BW. Special attention must be paid to the severity of PI. Fetal growth surveillance in the third trimester is warranted.

PMID: 30130989 [PubMed - as supplied by publisher]

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It Is Not Taboo: Addressing Sexual Function in Adults with Congenital Heart Disease.

Curr Cardiol Rep. 2018 Aug 22;20(10):93

Authors: Huang S, Cook SC

PURPOSE OF THE REVIEW: To review the current state of literature on sexual dysfunction in adults with congenital heart disease (ACHD).
RECENT FINDINGS: The prevalence of sexual dysfunction in ACHD is approximately 28%. Compared to age-matched cohorts, the prevalence of sexual dysfunction among ACHD cohorts demonstrates significant variability. ACHD have a lower rate of ever having sexual intercourse and often at a later age. Regardless of complexity, ACHD with sexual dysfunction have higher level of distress, decreased quality of life, and worse New York Heart Association classification. Patients, including heart failure and ACHD, treated with dual angiotensin receptor neprilysin inhibitor have reported improved sexual relationships. The prevalence of sexual dysfunction in ACHD patients is high and sexual dysfunction research in ACHD remains limited. Therefore, the relationship between sexual dysfunction and ACHD remains ill-defined. Cardiologists that participate in the care of these patients should proactively discuss sexual health and provide counseling and therapies to provide high-quality healthcare for ACHD.

PMID: 30136003 [PubMed - in process]

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Right partial anomalous pulmonary venous connection to the superior vena cava following the Warden procedure.

J Card Surg. 2018 Aug 22;:

Authors: Pavy C, Gavira N, Maminirina P, Baron O

BACKGROUND AND AIMS: Surgical repair of right partial pulmonary anomalous connection to the superior vena cava (SVC) with the Warden procedure can be complicated by SVC obstruction, pulmonary veins obstruction, and sinus node dysfunction. We review our 20 years of experience with Warden procedures for the repair of right partial pulmonary venous connection to the SVC.
METHODS: This was a single-center retrospective study of all patients (pediatric and adult) with right partial pulmonary abnormal venous connections who underwent a complete repair with the Warden procedure between 1997 and 2016. A total of 59 patients were included. The median age was 14 years (5 months to 61 years) and the median weight was 44 kg (4.9-92 kg).
RESULTS: The mean term follow-up was 7 (±5) years. No deaths were reported. One (1.7%) patient required a pacemaker implantation; two (3.4%) suffered from a SVC stenosis successfully treated with balloon dilatation and stent implantation; and eight (13.6%) patients had transient rhythm disturbances, one had junctional ectopic tachycardia, three had sick sinus syndrome, three had an atrial flutter, and one had an atrioventricular block. None suffered from pulmonary vein obstruction.
CONCLUSION: The Warden procedure is safe and can be performed with very low morbidity, mortality, rhythm disturbances, and decreased pulmonary and SVC obstructions.

PMID: 30136412 [PubMed - as supplied by publisher]

Pharmacological therapy in adult congenital heart disease: growing need, yet limited evidence.

Eur Heart J. 2018 Aug 21;:

Authors: Brida M, Diller GP, Nashat H, Strozzi M, Milicic D, Baumgartner H, Gatzoulis MA

Congenital heart disease (CHD) is the most common inborn defect. Due to advances in paediatric care, surgical, and catheter procedures the number of adults with CHD has grown remarkably in recent years. Most of these patients, however, have residua from their original operation/s and require life-long care, many of them are subjected to further haemodynamic and electrophysiological interventions during adulthood. While such re-do surgical or catheter interventions together with device therapy and transplantation play a key therapeutic role, increasingly, adults with CHD require drug therapy for late complications namely heart failure (HF), arrhythmias, pulmonary and systemic hypertension, thromboembolic events, etc. Unlike other cardiovascular areas, drug therapy in adult CHD is based on scarce clinical data and remains largely empiric. Consequently, pharmacological therapies are individualized to ameliorate patients' symptoms and/or degree of haemodynamic impairment. Thus far, recommendations have been difficult to make and formalized guidelines on drug therapy are lacking. We review herewith the rationale, limited evidence and knowledge gaps regarding drug therapy in this growing cardiovascular field and discuss pharmacotherapy options in specific conditions namely HF, arrhythmias, thrombosis, pulmonary arterial hypertension, contraception, and pregnancy.

PMID: 30137263 [PubMed - as supplied by publisher]

Genetic evaluation of patients with congenital heart disease.

Curr Opin Pediatr. 2018 Aug 22;:

Authors: Geddes GC, Earing MG

PURPOSE OF REVIEW: The aim of this study is to review genetics of congenital heart disease (CHD) with a focus on clinical applications, genetic testing and clinical challenges.
RECENT FINDINGS: With improved clinical care, there is a rapidly expanding population of adults, especially women, with CHD who have not undergone contemporary genetic assessment and do not understand their risk for having a child with CHD. Many patients have never undergone assessment or had genetic testing. A major barrier is medical geneticist availability, resulting in this burden of care shifting to providers outside of genetics. Even with current understanding, the cause for the majority of cases of CHD is still not known. There are significant gaps in knowledge in the realms of more complex causes such as noncoding variants, multigenic contribution and small structural chromosomal anomalies.
SUMMARY: Standard assessment of patients with CHD, including adult survivors, is indicated. The best first-line genetic assessment for most patients with CHD is a chromosomal microarray, and this will soon evolve to be genomic sequencing with copy number variant analysis. Due to lack of medical geneticists, creative solutions to maximize the number of patients with CHD who undergo assessment with standard protocols and plans for support with result interpretation need to be explored.This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

PMID: 30138133 [PubMed - as supplied by publisher]

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[Heart transplantation for the treatment of isolated left ventricular myocardial noncompaction. First case in Mexico].

Cir Cir. 2017 Nov - Dec;85(6):539-543

Authors: Zetina-Tun HJ, Careaga-Reyna G, Galván-Díaz J, Sánchez-Uribe M

BACKGROUND: Myocardial noncompaction of the left ventricle is a congenital cardiomyopathy characterised by left ventricular hypertrabeculation and prominent intertrabecular recesses. The incidence ranges from 0.15% to 2.2%. Clinical manifestations include heart failure, arrhythmias, and stroke. Prognosis is fatal in most cases. Heart transplantation is a therapeutic option for this cardiomyopathy, and few had been made worldwide.
CLINICAL CASE: The case is presented of a 20 year-old male with noncompacted myocardium of the left ventricle, who had clinical signs of heart failure. His functional class was IV on the New York Heart Association scale. He was successfully transplanted. Its survival to 15 months is optimal in class I New York Heart Association, and endomyocardial biopsies have been reported without evidence of acute rejection.
CONCLUSION: It is concluded that heart transplantation modified the natural history and improved survival in patients with this congenital heart disease.

PMID: 27773363 [PubMed - indexed for MEDLINE]