Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India.

OBJECTIVES: Congenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala to understand presentation of CHD, proportion of newborns with critical defects who receive timely intervention, outcomes at 1 month, predictors of mortality and barriers to timely management. METHODS: The congenital heart disease registry for newborns (≤28 days) in Kerala (CHRONIK) was a prospective hospital-based registry involving 47 hospitals from 1 June 2018 to 31 May 2019. All CHDs, except small shunts with a high likelihood of spontaneous closure, were included. Data on demographics, complete diagnosis, details of antenatal and postnatal screening, mode of transport and distance travelled and need for surgical or percutaneous interventions and survival were collected. RESULTS: Of the 1474 neonates with CHD identified, 418 (27%) had critical CHD, 22% of whom died at 1 month. Median age at diagnosis of critical CHD was 1 (0-22) day. Pulse oximeter screening identified 72% of critical CHD and 14% were diagnosed prenatally. Only 8% of neonates with duct-dependent lesions were transported on prostaglandin. Preoperative mortality accounted for 86% all deaths. On multivariable analysis, only birth weight (OR 2.7; 95% CI 2.1 to 6.5; p<0.0005) and duct-dependent systemic circulation (OR 6.43; 95% CI 5 to 21.8, p<0.0005) were predictive of mortality. CONCLUSIONS: While systematic screening, especially pulse oximetry screening, enabled early identification and prompt management of a significant proportion of neonates with critical CHD, important health system challenges like low use of prostaglandin need to be overcome to minimise preoperative mortality.

Anomalous Left Coronary Artery From Pulmonary Artery in a Baby With Pulmonary Atresia, Intact Ventricular Septum.

A baby with pulmonary atresia with intact ventricular septum and hypoplastic right ventricle, with suspected right ventricle to coronary communications, was operated on for placing an aortopulmonary shunt. Postoperatively, the baby deteriorated with features of myocardial ischemia. Postmortem examination revealed anomalous origin of left coronary artery from pulmonary artery that caused significant coronary ischemia on ligation of the ductus arteriosus. Although coronary anomalies, including right ventricle dependent coronary circulation, has been well described, this is the first report of anomalous origin of coronary artery from pulmonary artery in a baby with pulmonary atresia and intact ventricular septum.

Quantification of ventricular unloading by 3D echocardiography in single ventricle of left ventricular morphology following superior cavo-pulmonary anastomosis and Fontan completion - a feasibility study.

BACKGROUND: Three-dimensional echocardiography. (3DE) is comparable to cardiac magnetic resonance imaging for estimating ventricular volume in congenital heart diseases. However, there are limited data on estimation of ventricular volumes by 3DE in univentricular heart and change in ventricular volumes after surgical creation of cavopulmonary connection. We sought to quantify the unloading of the single ventricle of left ventricular. (LV) morphology by 3DE after superior cavopulmonary anastomosis. (SCPA) or Fontan operation over a period of 3 months and thereby derive a preliminary 3DE data set on this patient subset. PATIENTS AND METHODS: Eighteen patients with functional single ventricle of LV morphology, who underwent SCPA or completion of Fontan circulation, were included in the study. Volume of the ventricle was estimated by 3DE before surgery and after surgery. (in the early postoperative phase and 3 months after surgery), and indexed end-diastolic volume. (EDV), end-systolic volume. (ESV), and ejection fraction. (EF) were derived. RESULTS: Twelve patients underwent SCPA and six patients underwent staged completion of Fontan circulation. Before surgery, EDV was similar in both groups. There was a significant fall in EDV immediately after SCPA (from 48.3 ± 14.9 ml/m2 to 39.5 ± 12.3 ml/m2). However, EDV increased at 3 months' follow-up to 41.3 ± 10.5 ml/m2. There was no significant fall in EDV immediately after Fontan operation (47.2 ± 10.1 ml/m2-46.6 ± 14.2 ml/m2), but EDV continued to fall at 3 months of follow-up (44.7 ± 10. ml/m2). There was no significant change in ESV in either group, but EF fell significantly after SCPA. CONCLUSIONS: We provide preliminary information on 3DE volume data of single ventricle of LV morphology and the pattern of unloading after SCPA and Fontan operation. Immediate significant volume unloading occurred after SCPA which tended to catch-up after 3 months, whereas continued fall in ventricular volume with time was noted after Fontan.

Imaging the elusive anomalous origin of left coronary artery from pulmonary artery.

A 7-year-old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction.

An unusual thrombotic complication during percutaneous closure of atrial septal defect.

A case is described where intracardiac thrombus was observed at the junction of the superior vena cava and the right atrium during attempted closure of an atrial septal defect (ASD) using a device similar in design to the Amplatzer septal occluder (Heart R atrial septal occluder). Thrombosis was likely related to the multiple attempts required when deploying the device and the attendant prolonged procedural time. The patient underwent subsequent urgent surgical closure of the ASD.

Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy.

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.

Unusual coarctation-the PHACE syndrome: report of three cases.

OBJECTIVES: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome. RESULTS: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative. CONCLUSION: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.

Experiences with carotid endarterectomy at Sree Chitra Tirunal Institute.

BACKGROUND: Atherosclerotic carotid artery disease poses a grave threat to cerebral circulation, leading to a stroke with its devastating sequelae, if left untreated. Carotid endarterectomy has a proven track record with compelling evidence in stroke prevention. OBJECTIVES: a) To confirm that carotid endarterectomy (CEA) is safe and effective in preventing stroke at both short and long term. b) to demonstrate long term patency of internal carotid artery when arteriotomy repair is performed using autologous saphenous vein patch. MATERIALS AND METHODS: During ten years, from September 1997 to February 2008, thirty nine patients who underwent consecutive carotid endarterectomy at our institute, form the basis of this report. Their age ranged from thirty to seventy eight years, with a mean age of 56. There were four women in this cohort. Thirty seven patients were symptomatic with >70% stenosis and two were asymptomatic with >80% stenosis, incidentally detected. Imaging included Duplex scan and MRA for carotid territory and brain, and non-invasive cardiac assessment. Co-morbidities included smoking, hypertension, diabetes, and coronary artery disease. Carotid Endarterectomy was performed under general anaesthesia, using carotid shunt and vein patch arteriotomy repair. RESULTS: All the patients made satisfactory recovery, without major adverse cerebral events in this series. Morbidities included Transient Ischemic Attack (TIA) in two, needing only medications in one, and carotid stenting in the other. Minor morbidities included neck hematoma in two and transient hypoglossal paresis in three patients. Yearly follow-up included duplex scan assessment for all the patients. Two patients died of contralateral stroke, two of myocardial events and two were lost to follow up. Thirty three patients are well and free of the disease during the follow up of three to 120 months. CONCLUSION: Carotid endarterectomy provided near total freedom from adverse cerebral events and its catastrophic sequelae, leading to excellent outcome, both short as well as long term.