Low dose paracetamol for management of patent ductus arteriosus in very preterm infants: a randomised non-inferiority trial.

OBJECTIVE: To compare the efficacy of low dose-short course intravenous paracetamol with a conventional dose regimen for early targeted closure of patent ductus arteriosus (PDA). DESIGN: Single-centre, double-blinded, active controlled, randomised non-inferiority trial. SETTING: Level III neonatal intensive care unit in Western India. PATIENTS: Preterm infants <30 weeks of gestation requiring mechanical ventilation, or continuous positive airway pressure with FiO2 ≥0.35 and diagnosed with a haemodynamically significant PDA (hsPDA) at 18-24 hours of postnatal age. INTERVENTIONS: Low dose (10 mg/kg/dose 6 hourly for 72 hours) versus conventional dose (15 mg/kg/dose 6 hourly for 120 hours) intravenous paracetamol treatment. MAIN OUTCOME MEASURES: Comparison of the rates of ductal closure at completion of sixth postnatal day, using a prespecified non-inferiority margin of 20%. RESULTS: A total of 102 infants were enrolled. The median gestational age and birth weight of the included infants were 26.4 weeks and 830 g. At completion of the sixth postnatal day, closure of PDA was achieved in 92% of infants in the low dose group as compared with 94% of those in the standard dose group (risk difference: -1.6%, 95% CI: -11.6% to 8.5%, p=0.38). The rates of rescue therapies, adverse effects and other neonatal morbidities were comparable in both groups. CONCLUSION: In very preterm infants on significant respiratory support, low dose-short course intravenous paracetamol treatment was non-inferior to a conventional dosing regime of paracetamol for closure of hsPDA in the first week of postnatal age. Larger studies with narrow margins of non-inferiority are required to confirm our findings. TRIAL REGISTRATION NUMBER: CTRI/2017/10/010012.

Balloon Interrogation of Intervening Tissue: A Novel Method to Decide Strategy for Closing Multiple Atrial Septal Defects.

BACKGROUND: Two separate ostium secundum atrial septal defects are a challenging substrate for device closure due to lack of a well-described strategy or an adequately evaluated protocol. METHODS: This is a prospective study comprising 20 patients with 2 atrial septal defects who underwent device closure. All of them underwent balloon interrogation (BI) of the intervening tissue to decide 1- versus 2-device strategy. During BI, if the flow through both the defects could be stopped completely implying adequate mobility of the separating tissue, a single device strategy was used. The size of the device in this subset was determined by BI diameter. In case the flow persisted, 2 devices were used to close the defects separately. RESULTS: The mean age was 24±17 years. The main defect size was (mean 14.5 mm±SD 2.69 mm), whereas the second defect measured (mean 8.5±SD 3.02 mm). The tissue separating the 2 defects was measured (mean 6.1±SD 2.6 mm). In 15 of them, based on the BI results, a single device was used successfully to close both the defects without a residual shunt. In the remaining 5 patients, 2 devices were used. There were no complications during the procedure or at follow-up period of 41.9±16.9 months. CONCLUSIONS: BI in patients with 2 atrial septal defects is helpful in defining 1- versus 2-device strategies and in choosing the size of the device to be used. Nearly 3/4 of the patients may get away with a single device for closing both the defects successfully thereby decreasing the cost and complexity of the procedure.

Aortopulmonary window: Clues to fetal diagnosis.

Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge. A few echocardiographic signs can help to clinch the diagnosis.

Antenatal Assessment of Topsy-Turvy Heart With Crisscross Inlet.

Rare cardiac malpositions are faced with diagnostic challenges and may not follow set rules. The presence of more than one pathology simultaneously makes diagnosis challenging. The present case report describes antennal diagnosis topsy-turvy heart with crossed ventricular inlets. (Level of Difficulty: Intermediate.).

Transcatheter Closure of Iatrogenic Right Ventricular Perforation With Amplatzer Duct Occlude II.

Iatrogenic cardiac perforation is a major complication of cardiac intervention. Surgical correction of perforation is standard of care. We discuss a pacing wire induced right ventricular perforation during the coronavirus disease-2019 (COVID-19) pandemic. Open heart surgery was high risk due to age and COVID-related significant lung involvement. As a bailout measure, the perforation was successfully closed with transcatheter intervention. (Level of Difficulty: Advanced.).

Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect characterized by myocardial ischemia and ultimately scaring. The scar burden will determine eventual recovery of left ventricular function after corrective surgery. MATERIAL METHOD: All patients with proven diagnosis of ALCAPA and who underwent treatment at present centre were included. Detail echocardiography and cardiac magnetic resonance imaging (CMR) (delayed Gadolinium enhancement) was performed before and after surgery. RESULTS: There were 4 patients (3 females, age group 3 months to 3 yr, follow up 6 months to 20 months.) There was no peri operative mortality. All patients had significant improvement in symptom class and LVEF (increase of more than 10%) when evaluated at last follow up. Three patients had pre operative CMR and 3 post operative CMR. All patients had improvement in post operative LVEF, but >50% was observed only in one patient who had less than half thickness delayed gadolinium enhancement. The right coronary flow pattern were unique to disease. The left coronary flow pattern were had significant variation and could predict extent of scared myocardium. CONCLUSION: Ischemia in ALCAPA can lead to myocardial scarring even in early infancy. The recovery in left ventricular function is a closely related to scar burden. Coronary flow patterns are unique and give useful insight into disease process and natural history.

Unmasking the culprit: MAPCA masquerading as RV failure in post surgical correction of TOF.

Pulmonary regurgitation and right ventricular dilatation are major determinants of long-term follow up of surgically corrected tetralogy of Fallot. Occasionally, there are undiagnosed associated anomalies, which can exaggerate pulmonary regurgitation. The present report describes long-term effect of major aorto pulmonary collateral on right ventricular volume also a result of successful intervention. .

Bypassing the Pump.

We present a six-year follow-up of an infant who underwent a left subclavian artery to left anterior descending artery anastomosis for anomalous origin of the left coronary artery from the pulmonary artery, without the use of cardiopulmonary bypass. The clinical status, echocardiography, angiography, and exercise tolerance with treadmill test were found to be normal. This technique could be a viable alternative to the more established coronary translocation, with lesser morbidity, shorter hospital stay, and lower economic burden, especially in the developing world.

Very early onset traumatic hemolysis following mitral valve repair in a pediatric patient.

Hemolytic anemia following a mitral valve repair is a very rare complication, with only 70 cases reported worldwide. We present a case of an 8-year-old girl who presented with a very early onset hemolytic anemia following a mitral valve repair with ring annuloplasty, which necessitated a reoperation. The report also discusses the various mechanisms of traumatic hemolysis associated with valve repair and probable lessons learnt and ways to avoid this complication.

Color Doppler and pulse wave assessment of flow in anomalous origin of left coronary artery from pulmonary artery: Pre- and post-surgery.

Changes in left coronary artery flow pattern in anomalous left coronary from pulmonary artery can provide valuable insight into pathology and natural history of disease. We wish to discuss a case with pre and post operative left coronary flow pattern with mid term follow up.

An experience with off pump technique for repair of anomalous left coronary artery from pulmonary artery (ALCAPA).

BACKGROUND: Anomalous origin of left coronary artery from pulmonary artery is a very rare disease with incidence of one every 300,000 live births. It has a high mortality of 80% in the first year of life. This observational study summarized our experience using the technique of subclavian arterial bypass without the use of cardiopulmonary bypass (CPB) for treatment of this coronary anomaly in pediatric population. The study aims to revive an earlier technique, with modifications, as an alternative to the existing coronary translocation. METHODS: From 2009 till 2015, six consecutive infants were operated by a single surgeon using subclavian artery to left coronary artery bypass done off pump, to establish a two coronary circulation. RESULTS: Five patients had an improvement in their LV ejection fractions at the time of the last follow-up. Angiography done in two cases after 2 and 6 years after surgery revealed good flow in the left coronary artery and good growth in the length of subclavian artery. There was one surgical mortality in this series. CONCLUSIONS: The technique of off pump subclavian arterial bypass for anomalous origin of the left coronary artery from the pulmonary artery is a viable alternative to the existing standard technique of coronary translocation. It essentially eliminates extracorporeal circulatory support or a left heart bypass that may be needed after coronary translocation. Also, the advantage of avoiding CPB, both in economic terms and also the adverse effects related to use of bypass in this very sick hearts, cannot be disregarded.

Subvalvular apparatus and adverse outcome of balloon valvotomy in rheumatic mitral stenosis.

BACKGROUND: Balloon mitral valvotomy (BMV) is a well-established therapeutic modality for rheumatic mitral stenosis (RMS). However, there are chances of procedural failure and the more ominous post-procedural severe mitral regurgitation. There are only a few prospective studies, which have evaluated the pathogenic mechanisms for these major complications of BMV, especially in relation to the subvalvular apparatus (SVA) pathology. METHODS: All symptomatic patients of RMS suitable for BMV by echocardiographic criteria in a span of 1 year were selected. In addition to the standard echocardiographic assessment of RMS (Wilkins score and score by Padial et al.), a separate grading and scoring system was assigned to evaluate the severity of the SVA pathology. The SVA score was 'I', when none of the two SVAs had severe disease, 'II' when one of the two SVAs has severe disease, and 'III' when both SVAs had severe disease. With these scoring systems, the outcomes of BMV (successful procedure, failure, and post-procedural mitral regurgitation) were analyzed. Emergency valve replacement was performed depending on clinical situation, and in cases of replacement, the pathology of the excised mitral valves were compared with echocardiographic findings. RESULTS: Of the 356 BMVs performed in a year, 43 patients had adverse outcomes in the form of failed procedure (14 patients) and mitral regurgitation (29 patients). Forty-one among these had a SVA score of III. The sensitivity and specificity of the MR score was lesser than the SVA score (sensitivity 0.34 vs. 1.00, specificity 0.92 vs. 0.99, respectively). The mitral valvular morphology in 39 patients who underwent post-procedural valve replacements correlated well with echocardiography findings. CONCLUSION: It is important to assess the degree of SVA pathology in the conventional echocardiographic assessment for RMS, as BMV would have adverse events when both SVAs were severely diseased.

Transcatheter closure of large patent ductus arteriosus with severe pulmonary arterial hypertension: Short and intermediate term results.

OBJECTIVE: To assess the efficacy and safety of transcatheter closure (TCC) of patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PHT). BACKGROUND: TCC of small and moderate-sized PDAs is well established. However, there is a paucity of data on TCC of large PDA with severe PHT. METHODS: This is a retrospective observational study of 76 patients with large PDA and severe PHT who were referred for TCC. Multiple clinical and investigational parameters were evaluated to decide the reversibility of PHT. Following the TCC, patients were serially followed up to assess the efficacy and safety of closure and its impact on PHT. RESULTS: Of 76 patients, nine were found to have PDA with Eisenmenger's syndrome. Of remaining 67, two were thought to have irreversible PHT based on hemodynamic data obtained after balloon occlusion of the duct. Sixty five patients, who eventually underwent TCC of PDA, had a median age of 9.1 years (range 1 month to 40 years). The weight ranged between 2.5 to 62 kg (median 14 kg). The PDA size was 9.1 ± 4.6 mm. The mean systolic pulmonary artery pressure was 66.9 ± 15.3 mm Hg. Duct occluder was used in 63 and muscular ventricular septal defect closure device in 2. The follow up was available in 56 (86%) with a mean follow up period of 65 ± 34 months. All the patients had complete closure of the PDA at 6 months follow up. Mild obstruction of left pulmonary artery (n=3) and aortic isthmus flow (n=6) was noted at the time of discharge. During the follow up, partial or complete resolution of PHT was observed in all the patients in whom Doppler-derived right ventricular systolic pressure was recorded (available in 40 of 56 patients). CONCLUSIONS: TCC of large PDA with severe PHT and significant left to right shunt was found to be effective and safe in the short and intermediate term.

Aortic regurgitation following transcatheter closure of aortopulmonary window.

Natural history of aortic regurgitation (AR) following transcatheter closure (TCC) of intracardiac or aortopulmonary shunt needs to be explored. AR can appear immediately or later after TCC and may increase or regress. We describe a previously unreported AR development following successful TCC of aortopulmonary window.