Public-Private Partnership for Treatment of Congenital Heart Diseases: Experiences From an Indian State.

Background: Treatment of congenital heart disease (CHD), being the most common congenital anomaly, puts immense financial burden in low- and middle-income countries (LMICs) and contributes significantly to infant mortality. We report experiences of treatment of CHD in the Indian state of West Bengal by a public-private partnership (PPP) model. Methods: Under the Rashtriya Bal Swasthya Karyakram, the government of the state of West Bengal in India launched a program called the "Sishu Sathi Scheme" to provide free treatment to children who need heart surgeries, irrespective of economic status. Treatment was provided in selected private hospitals and some public hospitals in a reimbursement model where government compensated the hospitals. Data were collected on such procedures from 2013 to 2022 and analyzed. Results: A total of 27,844 patients with CHD received treatment under the Sishu Sathi Scheme from August 2013 to December 2022. The average number of patients per year was 3,093. Detailed data of procedures from January 2016 to December 2022 showed a total of 22,572 procedures (6,249 device interventions, 4,840 cardiac catheterizations, and 11,483 surgical interventions). The in-hospital mortality of surgical procedures and catheterization lab procedures were 5.2% and 0.9%, respectively. Conclusions: A large number of patients with CHD were successfully treated under a PPP in the state of West Bengal in India. In spite of its inherent challenges, this model is of special relevance in LMICs where access and affordability for treatment of CHD always remain a challenge.

Double left atrial appendage: A diagnostic dilemma.

We report a unique intraoperative finding of an additional double left atrial appendage (LAA) during an arterial switch operation with ventricular septal defect closure in a 4-month-old girl. Immediately after the procedure, a prolapsing mass within the left atrium (LA) on the transesophageal echocardiogram raised concerns of a possible thrombus. The LAA was clearly visible with a pressure monitoring line which was put intraoperatively. To investigate further, cardiopulmonary bypass was resumed, and the heart was arrested and explored. There was an appendage-like structure, separate from the one that had the pressure monitoring line, which was inverted inside. It was pulled out from outside clearly establishing a double LAA. This report illustrates an example of a diagnostic dilemma caused by a double atrial appendage which was invaginated into LA masquerading as a mass or thrombus.

Late presentation of aortopulmonary window: a contemporary series.

Late presentation of aortopulmonary window (APW) beyond infancy is uncommon and many of these cases are inoperable due to development of progressive pulmonary hypertension and Eisenmenger syndrome. Outcome data in this cohort is thus sparse and the aim of this study was to analyze the outcomes in patients with APW operable beyond 1 year of age. Between September 2016 and March 2020, in a single center, 12 consecutive patients older than 1 year, undergoing surgery for APW, were included in the study. The median age and weight at presentation were 7.5 years (interquartile range (IQR) 4-9.5) and 15 kg (IQR 11.7-19.5). Ten (83.3%) patients had type 1 APW (proximal type) and 2 (16.6%) had a type 2 APW (distal type). Eight (66.6%) patients had associated lesions. Transaortic patch closure of APW was done in all cases. Seven (58.3%) patients were extubated within 3.5 h of admission in intensive care. There were no early deaths or during follow-up. The median follow-up duration was 20.5 months (IQR 7.5-24), and all patients were in New York Heart Association (NYHA) class I at last follow-up. Follow-up echocardiography did not reveal any significant residual shunts necessitating any additional procedure and a consistent decrease in pulmonary artery pressures. Surgery in patients with APW beyond 1 year of age is possible in selected patients. The early and intermediate surgical outcomes in patients who remain operable are excellent.

Atypical presentation of complete heart block in children with pediatric inflammatory multisystem syndrome: A case series of two patients.

In the midst of the COVID-19 pandemic, we report two cases of children presenting with typical diagnosis of pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) who suffered from complete heart block requiring pacemaker placement which is an unusual presentation of PIMS-TS. We also compared it with the already existing data with similar manifestations. PIMS-TS is reported to occur in children with predominantly gastrointestinal symptoms, hemodynamic instability, and myocardial dysfunction. The implications of development of atrioventricular block during critical illness in PIMS-TS are yet unknown. Both patients had an otherwise normal cardiac structure and had no gastrointestinal symptoms but suffered complete heart block without any other identifiable etiology, both requiring temporary pacemaker placement. While one child recovered completely with medical management, the other child required permanent pacemaker placement. While we cannot be certain that COVID-19 was the cause, complete heart block appeared to be temporally related to COVID-19 infection in both patients, and hence, it is important for pediatricians to be aware of the potential manifestation of this disease.

A case of partial anomalous systemic venous drainage and perioperative detection of cerebral arteriovenous malformations.

We report a case of 8-year-old boy with unexplained desaturation and clubbing. Echocardiography showed anomalous drainage of right superior vena cava into left atrium. He did not have any neurological symptoms preoperatively. Some perioperative observations and subtle postoperative behavioral changes prompted us to investigate him further. He was found to have extensive cerebral arteriovenous malformations and hemiatrophy of brain.

Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up.

BACKGROUND: The standard first stage palliation for univentricular heart with unrestricted pulmonary blood flow (PBF) is surgical pulmonary artery (PA) banding for which the ideal age is within the first 8 weeks of life. This study aimed to look for the utility of PA band done beyond 3 months of age for patients presenting beyond the stipulated period. MATERIALS AND METHODS: This is a retrospective analysis of the outcome of twenty patients with single ventricle (SV) physiology with unrestricted PBF who presented late and were selected on the basis of clinical, radiological, and echocardiographic parameters for PA banding. RESULTS: The median age of the patients was 5.5 months (3.5-96 months), and the median body weight was 4.7 kg (3.2-22.0 kg). The patients were divided into three groups as follows: ten patients between 3 and 6 months of age (Group A), seven patients between 6 months to 1 year of age (Group B), and three patients > 1 year of age with additional features of pulmonary venous hypertension (Group C). The mean reduction of PA pressures following PA band was 60.9%, 48.8%, and 58.3% and the mean fall in oxygen saturation was 10.4%, 8.0%, and 6.6% in the three groups, respectively. The postoperative mortality rate was 10%. The mean follow up duration was 13.5 months (7-23 months). There was a statistically significant improvement in weight for age Z scores following PA band (P = 0.0001). On follow up cardiac catheterization, the mean PA pressures were 16.6 (±3.6), 22.7 (±5.7), and 33.3 (±12.4) mmHg, respectively, in the three groups, and the mean pulmonary vascular resistance index was 1.86 (±0.5), 2.45 (±0.7), and 3.5 (±1.6) WU.m2, respectively. Subsequently, seven patients in Group A, three patients in Group B, and one patient from Group C underwent successful bidirectional Glenn (BDG) surgery. CONCLUSIONS: Late PA band in selected patients with SV physiology can have definite benefit in terms of correction of heart failure symptoms and subsequent conversion to BDG and can potentially change the natural history of disease both in terms of survival and quality of life.

Percutaneous closure of moderate to large perimembranous ventricular septal defect in small children using left ventricular mid-cavity approach.

AIM & OBJECTIVE: To report mid-term follow-up result of transcatheter closure of perimembranous Ventricular septal defect (VSD) in children weighing less than 10 kg using Amplatzer Duct Occlude-I (ADO-I) by left ventricular (LV) mid-cavity approach. MATERIAL & METHOD: This is retrospective review of 35 children weighing less than 10 kg with moderate to large perimembranous VSD who were selected for transcatheter closure of VSD using ADO-I in between October 2016 to September 2018. Mean age was 2.08 ± 0.67 years (mean ± SD) and mean weight was 7.2 ± 1.2 kg (mean ± SD). Procedure was done by crossing the VSD from right ventricular side instead of using the standard approach by forming arterio-venous loop. Average fluoroscopic time was 9.2 ± 2.9 min (mean ± SD) and mean procedure time was 34.1 ± 13.1 min (mean ± SD). Mean follow-up period was 8.7 months (3-12 months) RESULT: Device closure was successfully done in 32 patients. There was device induced aortic regurgitation (AR) in one case who was sent for surgery. One child was found to have complete heart block on first post-procedure day requiring permanent pace-maker implantation. During follow up none of them had any residual VSD, rhythm disturbance, AR and left or right ventricular outflow obstruction. CONCLUSION: Device closure can be successfully done in moderate to large perimembranous VSD using left ventricular mid cavity approach in small children. LV mid-cavity approach has advantages in terms of lesser hemodynamic instability, lesser fluoroscopy and lesser chance of device induced AR than standard technique particularly in smaller children.

Congenital right ventricular diverticulum: An atypical presentation.

Congenital ventricular diverticulum is a rare congenital malformation of the heart. It remains mostly asymptomatic unless associated with other congenital cardiac defects. We present a case of a 4-month-old child who had dextrocardia, large inlet ventricular septal defect, amounting to single ventricle, and severe pulmonary artery hypertension. There was a right ventricular diverticulum which passed through the foramina of Morgagni to give rise to a pulsating lump in the epigastrium. Right ventricular diverticulum presenting with a pulsating mass in the abdomen is a rare entity. The confirmation of diverticulum was made with histopathology.

Percutaneous salvage interventions in the Glenn circuit: a case series.

BACKGROUND: In majority of children bidirectional Glenn shunt is a safe and efficacious procedure with minimal post-operative issues. Rarely, there may be dysfunction in the Glenn pathway due loss of anatomical integrity or derangements in normal physiological or hemodynamic milieu. We report 4 cases in the last 3 years (2016-2019) where complications in the Glenn circuit led to serious consequences requiring transcatheter interventions. CASE PRESENTATION: Two of our patients presented with frank features of superior vena cava syndrome. One of them had right Glenn anastomotic site narrowing leading to severe obstruction along with significant left pulmonary artery origin stenosis. The other child had excessive antegrade flow impeding normal Glenn flow leading to superior vena cava syndrome. The next child in our series was initially lost to follow-up after bidirectional Glenn surgery. Later on, this child was noted to have discontinuous left pulmonary artery with perfusion only to the right lung from the Glenn. The remaining child described in this series had developed a large tortuous venous collateral post Glenn shunt leading to severe cyanosis. All the above children needed prompt percutaneous interventions to revert back to their basal state. On follow-up, the benefit was sustained in all. CONCLUSIONS: Percutaneous intervention procedures often provide a successful bailout option in various complicated situations post Glenn surgery with reasonable efficacy and safety.

Novel use of Konar-multifunctional occluder.

INTRODUCTION: Lifetech Konar-multifunctional occluder is a novel device which is primarily used for the closure of ventricular septal defects. Being "multifunctional", the occluder has the potential to be useful in various structural cardiac defects. MATERIALS AND METHODS: We share our retrospective review from two centres regarding non-conventional usage of multifunctional occluders in CHD. Eight patients who underwent interventions using multifunctional occluders for lesions other than ventricular septal defects between March 2019 to September 2019 were included in the study. The patients were analysed based on demography, the size and type of lesion, procedural success, and development of complications. All patients were followed up in the outpatient department for a minimum period of 6 months. RESULTS: The median age and weight of the cohort were 3.2 years and 9 kg, respectively. Six patients had patent ductus arteriosus, while one patient had aorto-pulmonary window and one had a coronary arterio-venous fistula. The sizing of the occluders and the procedural approach were based on the underlying pathology. The most commonly used occluder was 6 × 4 mm variant. One patient had successful implantation but had significant intra-device residual flow and was thus replaced by a different occluder. There were no major complications, nor any incidences of device embolisation or malposition. On follow-up, all patients had uneventful course. CONCLUSION: Konar-multifunctional occluder can be safely used in lesions other than ventricular septal defects, when needed under specific circumstances. Its unique characteristics make it a versatile choice in a variety of cardiac lesions.

Percutaneous rescue therapy in a child with blocked cavo-pulmonary shunt.

A 7-month-old infant presented with bilateral blocked cavo-pulmonary anastomosis within 2 months of surgery. Due to extreme haemodynamic instability, surgical options were abandoned and rescue intervention from left jugular line was planned. Acute thrombosis of the left-sided Glenn was noted with significant anastomotic narrowing. Successful rescue thrombolysis was done using recombinant tissue plasminogen activator (Alteplase) along with balloon dilatation of the attenuated segments.

Transcatheter closure of ventricular septal defect in aortic valve prolapse and aortic regurgitation.

OBJECTIVE: To report intermediate follow-up result of transcatheter closure of ventricular septal defect (VSD) in presence of aortic valve prolapse (AVP) with or without aortic regurgitation (AR). METHOD: This is a retrospective review of 19 patients with VSD with AVP with AR who underwent transcatheter closure in between September 2011-July 2014. Mean age was 8 years (1-16 years, standard deviation [SD] 4.08 years) and mean weight was 26.03kg (9-81.5kg, SD 16.57kg). Among them 2 had subarterial VSD, 6 had subaortic VSD and 11 had perimembranous VSD. All of them had mild AVP and 13 of them had AR (trivial or mild). Median VSD size was 4.3mm (4-6mm). Transcatheter closure was done either by retrograde technique using the Amplatzer Duct Occluder-II in 17 patients or antegrade technique using the Duct Occluder-I in 2 cases. Mean follow-up period was 18 months (12-36 months). RESULT: Immediate major complications were encountered in 2 (10.5%) cases. Significant aggravation of device related AR was seen in one case & device embolised to right pulmonary artery in another case and both of them were managed surgically. During follow up, 1 child had significant additional VSD requiring device closure. One child developed moderate AR, requiring surgery. None of the other had shown any increase in severity of AR. CONCLUSION: Device closure of VSD in presence of mild AVP and mild AR appears to be safe. Longer follow-up is necessary to draw final conclusion.

Complete heart block following transcatheter closure of perimembranous VSD using amplatzer duct occluder II.

Long term follow up studies have shown that transcatheter closure of perimembranous Ventricular Septal Defect (PmVSD) can be done safely and successfully. One of the most serious complications of VSD device closure is complete atrioventricular block which has been reported in 3-18% is various studies following VSD device closure. Amplatzar Duct Occluder II (ADO-II) device is now commonly being used for closure of small to moderate sized PmVSD. Complete heart block has not been reported with ADO-II device. We are reporting 2 cases of complete heart block following transcatheter closure of PmVSD using ADO-II device where sinus rhythm was established following early surgical removal of the device.