The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales

Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality.Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality.Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.

Prenatal diagnosis of double outlet right ventricle with intact ventricular septum in two foetuses

We describe two cases of an unusual variant of double outlet right ventricle with intact ventricular septum diagnosed prenatally and confirmed by foetal autopsy in a case. The first case had mitral valve atresia, slit-like left ventricle, and normally related great arteries. The second case had mitral valve atresia, hypoplastic left ventricle, parallel outflows with an interrupted aortic arch.

Silent in situ thrombus within a hypoplastic left ventricle in a teenager with Fontan palliation

Thrombosis, especially in the Fontan pathway, is one of the major concerns in patients who underwent Fontan surgery, with reported prevalence of 5–33%. We report a case of thrombus in a rudimentary left ventricle in teenager with no arrhythmia or neurological complications. We also report the special concerns of silent thrombus and role of cardiac MRI in diagnosing an intracardiac thrombus.

Surgical Outcomes of Biventricular Repair for Hypoplastic Left Ventricle With Congenital Mitral Valve Stenosis

Objective: Surgical outcomes of biventricular repair for hearts with hypoplastic left ventricle with congenital mitral valve stenosis are described. Serial changes of left ventricular geometry and clinical features after biventricular repair were reviewed. Methods: Eight patients with hypoplastic left ventricle and congenital mitral valve stenosis who underwent first surgical intervention for biventricular circulation in neonatal or infantile period between 2001 and 2014 comprise the study population. Serial change in left ventricular end-diastolic diameter, left ventricular mass index, and relative wall thickness after biventricular repair were evaluated by two-dimensional echocardiography. Results: The median Z-scores of left ventricular end-diastolic diameter and mitral valve diameter before the first surgical intervention were −3.0 (range, −4.8 to −2.0) and −1.0 (−2.9 to 2.1), respectively. Mitral valves were surgically treated in five patients; they were replaced in two and repaired in three patients. Left ventricular end-diastolic diameter Z-score at five years after biventricular repair was 0.1 (−3.0 to 1.0), which was significantly larger than before first surgical intervention ( P = .005). Left ventricular mass index, on the other hand, did not change, but relative wall thickness significantly decreased ( P = .009). Postoperative catheter study showed pulmonary hypertension with high left ventricular end-diastolic pressure in more than half of survivors. Conclusions: Left ventricle increased in size after the biventricular repair with appropriate mitral valve procedures before progression of pulmonary hypertension. Left ventricular mass, however, did not accompany the increase. Some patients may have suffered from mild, but certain restrictive left ventricular physiology and subsequent pulmonary hypertension as the result of abnormal remodeling process of the myocardium.

Profile of Fetal Echocardiography in a Tertiary Cardiac Centre of Nepal

Aims: To analyze the fetal echocardiographic diagnosis.Methods: A descriptive study of fetal echocardiographic diagnosis at Shahid Gangalal National Heart Centre between October 2016 to September 2017. Results: Total of 615 fetal echocardiography was performed in 607 pregnancies. Intrauterine heart disease noted in 79 cases. Echogenic intracardiac foci was the most common abnormality (6%) followed by VSD (1.3%), fetal arrhythmia (1.1%), pericardial effusion (1.6%), cardiomyopathy (0.3%), hypoplastic left ventricle (0.6%), DORV (0.3%) and tricuspid atresia (0.4%). Similarly the most common referral for fetal echocardiography was for maternal disorder mostly diabetes mellitus.Conclusion: Fetal echocardiography is an important tool for the antenatal diagnosis of congenital heart defects. Appropriate timing and judicious use increases the sensitivity and improves the perinatal outcome of newborns with congenital heart disease

Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children’s Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children’s Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle—46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.