Choosing an appropriate size valve for transcatheter pulmonary valve implantation in a native right ventricle outflow tract.

Introduction: Transcatheter pulmonary valve implantation has been an effective treatment for dysfuntional right ventricular tract outflow tract (RVOT). Defining a landing zone before the intervention is crucial in patients with native RVOT. Improper sizing and undefined landing zone will lead to embolization. Methods: It is a retrospective observational study from August 2020 to December 2020 in native RVOT. Three patients who had significant Right ventricle dilatation were analyzed. The multi-slice computed tomography (MSCT) with magnetic resonance imaging and angiography data of all patients before the procedure were analyzed. All patients underwent an angiogram in the same sitting, before the procedure to assess the landing zone, valve diameter as well as the risk for coronary compression. We chose a valve based on valve area 23%-25% more than the area at the waist during balloon sizing. Results: All three patients underwent successful valve implantation. Valve sizes used were 27.5 mm in one and 32 mm in the other two. The mean RVOT gradient postprocedure was 11.5 mm Hg and pre procedure was 43 mmHg. There were no complications during the procedure or at a mean follow-up of 3.6 months. Conclusion: The balloon sizing gives the true narrowest diameter in comparison with MSCT, and increasing this area by 23%-25% will give the appropriate valve size for successful implantation.

Successful management of a neonate with antenatally detected mature intrapericardial teratoma.

Intrapericardial teratoma is a germ-cell tumor that typically arises from the base of the heart and usually diagnosed in the fetal or neonatal period. Although benign, these tumors can be massive in size causing direct compression of the heart. Life-threatening complications such as fetal hydrops, cardiac failure, superior vena cava syndrome, and cardiac tamponade caused by these teratomas have been reported. Early surgical excision is curative. We present the images of a mature intrapericardial teratoma diagnosed in an asymptomatic neonate. The neonate was managed successfully by elective surgical excision.

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.

Cardiac surgery-associated acute kidney injury in a developing country: Prevalence, risk factors and outcome.

Little is known about cardiac surgery-associated acute kidney injury (CS-AKI) in children in developing regions of the world. The study aimed to determine the prevalence of CSAKI, associated factors and its impact on mortality and utilization of hospital services. The hospital records of children aged 0-17 years who underwent CS at an Indian hospital were reviewed. CS-AKI was defined as a rise in serum creatinine of ≥0.3 mg/dL in any 48 h and or by urine output <0.5 mL/kg/h for an 8-h period in the first five days after CS. The study included 323 children with a median age of one year (0.04-17), of whom 22 (6.8%) were neonates and 18.3% had a single ventricle. About 60% of the children had Risk Adjusted Congenital Heart Surgery-I category 1 or 2 interventions. CS-AKI occurred in 39 children (12.1%). Factors associated with CS-AKI were sepsis and intraand post-operative hypotension. In-hospital mortality was six-fold higher in children who developed CS-AKI. CS-AKI was associated with two to three days more of mechanical ventilation and Intensive care unit stay. CS-AKI occurs in children in developing countries, but at a lower frequency mainly due to the predominance of post-neonatal children undergoing less-complex CSs. CS-AKI was associated with higher in-hospital mortality and increased utilization of hospital services. Factors associated with CS-AKI included intraand post-operative hypotension and sepsis.

Cruciate Fenestration in Ventricular Septal Defect Patch for High-Risk Patients With High Pulmonary Vascular Resistance.

Late presentation of patients with large ventricular septal defect (VSD) and elevated pulmonary vascular resistance (PVR) is not uncommon in developing countries. Surgical VSD closure in these patients carries risks of persistent pulmonary hypertension, right ventricular failure, and mortality. Several techniques for creation of valved patches or fenestrated patches have been developed to address these issues. We have successfully used a simple and easily reproducible technique in which a cruciate fenestration is created in the patch used for VSD closure.

Hybrid intraoperative pulmonary artery stenting in redo congenital cardiac surgeries.

OBJECTIVE: Reconstruction of branch pulmonary arteries (PAs) can be challenging in redo congenital heart surgeries. Treatment options like percutaneous stent implantation and surgical patch angioplasty may yield suboptimal results. We present our experience with hybrid intraoperative stenting which may be an effective alternative option. METHODS: We retrospectively analyzed data of all patients with PA stenosis who underwent intraoperative PA branch stenting in our institution between January 2011 and December 2012. RESULTS: Ten patients [6 females, median age 10 (1.4 to 37) years], underwent hybrid stenting of the PA. Primary cardiac diagnoses were pulmonary atresia with ventricular septal defect (VSD) in three patients, pulmonary atresia with intact ventricular septum in two, Tetralogy of Fallot (TOF) in one, Double outlet right ventricle (DORV) with pulmonary stenosis (PS) in one, complex single ventricle in two and VSD with bilateral branch PA stenosis in one patient. Concomitant surgeries were revision/reconstruction of RV-PA conduit in 4, Fontan completion in 4, repair of TOF with conduit placement in 1 and VSD closure in 1 patient. The left PA was stented in 7, the right in 2 and both in 1, with a total of 11 stents. There were no complications related to stent implantation. Two early postoperative deaths were unrelated to stent implantation. At mean follow-up period of 14.8 (12-26) months, stent position and patency were satisfactory in all survivors. None of them needed repeat dilatation or surgical reintervention. CONCLUSION: Hybrid stenting of branch PA is a safe and effective option for PA reconstruction in redo cardiac surgeries. With meticulous planning, it can be safely performed without fluoroscopy.

Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect.

Coronary artery to pulmonary artery communications (CAPAC) are an important source of pulmonary blood flow in approximately 10% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair. We present two cases of PA-VSD with CAPAC, one in a 7-year-old child and the other in a 33-year-old adult. The method of occlusion of these communications could be either surgical or catheter based.

Do preoperative haemodynamic data and reactivity test predict the postoperative reversibility of pulmonary arterial hypertension in patients with large ventricular septal defect and borderline operability?

BACKGROUND: Decisions to operate on patients with shunt lesions presenting late with severe pulmonary arterial hypertension (PAH) and borderline operability are often not based on precise cut off values of haemodynamic data owing to paucity of studies. OBJECTIVE: To assess the reliability of the preoperative haemodynamic data and reactivity test in predicting the postoperative reversibility of PAH in patients with isolated large ventricular septal defects (VSDs) and borderline operability. PATIENTS AND METHOD: Between 2004 and 2010, 30 patients underwent VSD closure surgically; no early deaths occurred. Twenty-six patients were followed up regularly (mean 39.6±16 months) and one late postoperative death occurred. Fourteen patients who had been followed up for at least 1 year postoperatively underwent cardiac catheterisation. RESULTS: There were 3 responders (asymptomatic patients with pulmonary vascular resistance (PVR) index <3 WU.m(2)) and 12 non-responders. The following were lower among responders: mean age at surgery (3.2±0.42 vs 11.55±3.29 years, p=0.227), mean baseline PVR index (3.69±0.8 vs 10.57±9.1, p=0.204), average resistance ratio (RR=0.25±0.01 vs 0.59±0.25, p=0.049) and ratio of pulmonary and systemic mean pressures (PAm:SAm ratio) (0.70±0.009 vs 0.87±0.118, p=0.003). CONCLUSIONS: Preoperative 'base line' PAm:SAm and RR appear to be better predictors of postoperative outcome than other baseline parameters. Preoperative reactivity test had no significant role in predicting postoperative reversibility of PAH at mid-term.

Transcatheter interruption of large residual flow after device closure of "Type A" patent ductus arteriosus.

We report a case of 3-year-old girl who had persistence of large residual flow following transcatheter closure of a 6 mm 'Type A' patent ductus arteriosus using a 12 × 10 mm duct occluder. Angiography revealed a large left-to-right shunt coursing through and exiting around the implanted device. Near total abolition of the residual shunt was achieved by initial implantation of an embolization coil within the duct occluder and subsequently an Amplatzer duct occluder (ADO II) adjacent to the duct occluder. This challenging case describes an additional technique of abolishing a large residual flow in and around a Nitinol duct occluder device.

Mycotic aneurysm complicating a covered stent implanted for coarctation of the aorta in a child.

A mycotic aneurysm associated with a covered stent in the thoracic aorta of a 12-year-old child was successfully managed by excision and replacement with aortic homograft. On follow-up, there was unobstructed flow through the homograft. This case highlights the need for high index of suspicion for mycotic aneurysm and prompt surgical intervention in children with coarctation of aorta who present with features of infective endocarditis.

Unusual combination of hypoplastic left ventricle, atrioventricular septal defect with restrictive ventricular septal defect, and common arterial trunk.

We describe rare cases of common arterial trunk (truncus arteriosus communis) with unbalanced atrioventricular septal defect, left ventricular hypoplasia, and restrictive ventricular septal defect. The embryology, hemodynamics, and the clinical implications of this complex combination are discussed.

The curious case of a button which led to the needle.

Foreign bodies in the heart are uncommon in children. These are often removed even if asymptomatic to prevent complications like erosion, embolization, bleeding, thrombosis, and endocarditis. We report the case of a one-and-a-half-year-old child with a hypodermic needle in the heart which was found incidentally and removed successfully by surgery.

Fontan conversion with concomitant arrhythmia surgery for the failing atriopulmonary connections: mid-term results from a single centre.

OBJECTIVES: Classical Atriopulmonary Fontan connections tend to fail in the long term due to progressive anastomotic site obstruction, right atrial enlargement, and refractory atrial arrhythmias. Conversion to total cavopulmonary connection with concomitant arrhythmia surgery is a promising treatment but optimal timing of the procedure remains controversial. METHODS: Between the years 2002 and 2009, 15 patients with a median age of 26.2 (12-43) years underwent Fontan conversion operation with concomitant arrhythmia surgery. All were symptomatic and 14 out of the 15 patients had refractory arrhythmias. The duration of pre-operative arrhythmia and the outcome of surgery were correlated to study the impact of delay in surgical intervention on post-operative survival and arrhythmia control. RESULTS: There were two patients who died in the early post-operative period (13.3%). At the mid-term follow-up, 53 (20-86) months, late atrial arrhythmias had recurred in two of the 13 surviving patients (15.30%) and one patient developed late sinus node dysfunction. The need for anti-arrhythmic drugs decreased considerably from 93.5% to 15.3% on mid-term follow-up. There was no late death or need for cardiac transplantation. The duration of arrhythmia before surgery was prolonged for more than 10 years in patients who died as well as in those who had complications like late recurrence of arrhythmias, dependence on anti-arrhythmic medications, and worsening of ventricular dysfunction. CONCLUSIONS: Fontan conversion is a well-established treatment option for salvaging the failing atriopulmonary connections. Concomitant arrhythmia surgery effectively resolves the refractory atrial arrhythmias and improves survival, but we need to optimise the timing of Fontan conversion to improve the long-term outcome.

A gratifying surgical repair of anomalous left coronary artery from pulmonary artery: three decades of follow-up.

Establishing a two coronary system is the best treatment option for anomalous origin of left coronary artery from the pulmonary artery (ALCAPA). We describe a case of ALCAPA with three decades of follow up after an end-to-end anastomosis of left subclavian artery and left coronary artery with mitral valve replacement. This technique of establishing a two coronary system was done in an era when coronary transfer technique was not established and myocardial protection techniques were not available. This case is also the longest follow up of prosthetic mitral valve replacement in a child with ALCAPA.

Anatomically corrected malposition of great arteries.

Anatomically corrected malposition of great arteries (ACMGA) is a rare form of congenital heart disease in which the great arteries arise above the anatomically correct ventricles but have abnormal spatial relationship. We report the case of a 26-year-old female with ACMGA and tunnel type of subaortic obstruction. The abnormal relationship and segmental arrangement necessitates systematic approach in evaluation for proper diagnosis and surgical repair. This unusual case is reported for its rarity and to highlight the need for awareness to differentiate it from other more common conditions.