Background : Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim : We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods : In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental “delay” and “no delay” groups. Results : Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental “delay” and “no delay” groups (P = 0.04). Conclusion : Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

Objective : Pulmonary vein stenosis (PVS) is an emerging cause of pulmonary hypertension in preterm infants. It is an often lethal condition with poor long.term prognosis and high mortality. Previous work suggests an association between necrotizing enterocolitis (NEC) and PVS, supporting a possible role for inflammatory processes due to gastrointestinal (GI) pathology as an associated risk factor for PVS. Study Description : We performed a matched case–control study where infants with PVS were matched for gestational age with infants without PVS. Hospital records were reviewed for prior history of NEC or other gut pathology. Results : Twenty-four PVS patients were matched with 68 controls; 63% of patients (15/24) had prior GI pathology as opposed to 19% (13/68) of controls. The GI pathology group had a significantly higher growth restriction and C-reactive protein. The mean gradient across the pulmonary veins was higher in the gut pathology group versus controls, as was mortality (29% vs. 9%). Conclusions : The previously described association between PVS and intestinal pathology was further strengthened by this study. The presence of GI pathology should lead to early surveillance and intervention for PVS.

Background : Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods : Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed (n = 109), those with technically limited images for Qp/Qs calculation (n = 11) and those with time interval between TTE and cath >60 days were excluded (n = 14). Qp/Qs was calculated from stored clips by previously described methods and correlated with those obtained by oximetry. Patients were subdivided into two age groups <21 (Group 1) and ≥22 years (Group 2). TTE and cath methods for Qp/Qs estimation were compared using paired t-test, Pearson's correlation coefficient, and Bland–Altman plots. Results : Eighty-four subjects met inclusion criteria (age range 3–78 years). Group 1 n = 35; median age 10 years; Group 2 n = 49; median age 49 years. Transthoracic echocardiogram was performed 19.5 ± 15 days prior to cath. Mean Qp/Qs derived by cath and TTE were 2.09 ± 0.9 versus 2.54 ± 1.2 (P < 0.0001). Overall correlation was poor between the methods (r² = 0.32, P < 0.0001) and continued to be poor for Groups 1 and 2 (r₂ = 0.24, P = 0.003 and r₂ = 0.40, P < 0.0001 respectively). Bland–Altman plots demonstrated poor agreement between the predetermined limits of agreement (-0.5–1.5). Conclusion : Transthoracic echocardiography estimated Qp/Qs, although routinely utilized in clinical practice, has poor correlation and agreement with oximetry-derived Qp/Qs. The test performs poorly in all age groups in detecting a hemodynamically significant shunt and tends to overestimate the degree of left to right shunt.

Background : We evaluated our early experience of cardiac procedures in children with congenital heart defects (CHD) after asymptomatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, to understand whether recent reverse transcription-polymerase chain reaction (RT-PCR) positivity had a negative impact on their postprocedural recovery and outcomes. Materials and Methods : In this retrospective observational study, all patients with CHD who underwent cardiac surgery or transcatheter intervention at our institution between March 2020 and June 2021 who were detected to have asymptomatic SARS-CoV-2 infection on routine RT-PCR were included. Details of the cardiac procedure and postprocedural recovery were reviewed and compared with RT-PCR-negative patients who concurrently underwent similar cardiac surgeries or interventions at our center. Results : Thirteen patients underwent cardiac surgery after recent SARS-CoV-2 positivity after a mean interval of 25.4 ± 12.9 days. One patient expired with multiorgan dysfunction and systemic inflammatory response with elevated D-dimer, serum Ferritin, C-reactive protein, and significant ground-glass opacities on chest radiograph. Another patient developed spontaneous thrombosis of the infrarenal abdominal aorta, bilateral iliac arteries, and bilateral femoral veins, requiring low-molecular weight heparin postoperatively. This patient's postoperative recovery was also prolonged due to lung changes delaying extubation. All other patients had uneventful postprocedural recovery with intensive care unit and hospital stays comparable to non-SARS-CoV-2-infected patients. Conclusions : From our early experience, we can surmise that an interval of 2–3 weeks after asymptomatic SARS-CoV-2 infection is adequate to undertake elective or semi-elective pediatric cardiac surgeries. For patients requiring emergent cardiac surgery prior to this interval, there is potentially increased risk of inflammatory and/or thrombotic complications.

Background : Vasoactive medications are frequently used in the preoperative stage to balance the pulmonary and systemic blood flow. However, not much is known about the effects of these agents during this stage. Aims : The primary objective of this study was to characterize the acute effects of vasoactive medications in children with parallel circulation before either the hybrid or Norwood procedure. Setting and Designs : This is a single-center, cross-sectional, retrospective study. Methods : Hemodynamic and systemic oxygen delivery data were captured from patients' vital signs, arterial blood gases, near-infrared spectroscopy monitoring (NIRS). Data for each patient were collected before the initiation of a vasoactive medication and again 6 h after. Statistical Analysis : Data were analyzed using paired t-tests, and analysis of covariance. Results : A total of 139 patients were identified. After data extraction the following patients were included before the initial intervention: 7 were on milrinone, 22 were on dopamine, and 17 were on dobutamine. Dopamine and dobutamine were found to significantly increase systolic blood pressure. Only dopamine increased pH (mean difference 0.04), decreased paCO2 (mean difference -7.1), decreased lactate (mean difference -0.6 mmol/L), and decreased in bedside Qp: Qs (mean difference -7.5) after continuous infusion for 6 h. Milrinone was not associated with any significant hemodynamic change. Conclusion : In this study, dopamine was independently associated with improvement in markers of systemic oxygen delivery 6 h after initiation. Dobutamine and dopamine were associated with increased in blood pressure. Well-powered studies are required to detect changes in lactate and NIRS.

Objectives : We aimed to compare the performance of pulse-oximetry screening in detecting nonductus-dependent cyanotic congenital heart defects (CCHDs). Methods : In a prospective cross-sectional study, we recorded post ductal saturation of neonates (<48 h old) born at a community hospital in northern India. Subsequently, all underwent clinical examination and echocardiogram by a trained cardiologist. A saturation <95% was considered a “failed” screen. Results : Ten neonates were identified to have nonductus-dependent CCHD on echocardiogram, five of whom had passed pulse-oximetry screen. This translated to a sensitivity of 50% (95% confidence interval [CI] 23.7%–76.3%) and a positive predictive value of 0.08 (95% CI 0.03–0.2), both of which were significantly less compared to that in ductus-dependent congenital heart defect. Conclusions : Up to half of the nonductus-dependent CCHD may be missed if screened only using pulse oximetry. Parents should not be reassured regarding the absence of CCHD only based on a “pass” in pulse-oximetry screening.

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea–orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

Segmental pulmonary arterial hypertension occurs when the right and left pulmonary arteries are nonconfluent and receive blood supply from different sources. High blood pressure confined to limited lung segments may accelerate progression of pulmonary vascular resistance. Calculation of segmental vascular resistance and assessment of operability in such situations are done after integrating catheter hemodynamics, magnetic resonance imaging techniques, or perfusion scintigraphy. When an isolated pulmonary artery perfused by a restrictive ipsilateral arterial duct is associated with a large nonrestrictive contralateral arterial duct connected to the other pulmonary artery, leading to unilateral pulmonary arterial hypertension and features of high vascular resistance, it offers unique challenges to decision-making.

We report treating a term neonate with tuberous sclerosis and giant rhabdomyomas who presented with incessant supraventricular tachycardia with Everolimus. The treatment was efficient in reducing tumor size and assisted as an adjunct therapy in controlling arrhythmia and limiting preexcitation. Treatment was challenged by difficulty to achieve stable drug level and limited by neutropenia as a serious side effect.

Congenital JET (junctional ectopic tachycardia) is a rare and often difficult to treat tachyarrhythmia in young infants. The addition of Ivabradine to standard Congenital JET therapy has been shown to improve arrhythmia control. However, Ivabradine has not been reported as a single drug in the control of congenital JET. We report a pre-term neonate in whom Ivabradine monotherapy was successful in treating congenital JET.

The diagnosis of long QT syndrome (LQTS) in utero presents many challenges for clinicians, and there is high risk for intrauterine fetal demise as life-threatening arrhythmias develop secondary to QT prolongation. We describe a challenging case of a fetus presenting with sinus bradycardia and second-degree atrioventricular block with episodes of ventricular tachycardia. A prenatal diagnosis of LQTS was suspected given the fetal echocardiographic findings of a short ventricular relaxation time, due to extremely prolonged refractory period. The patient was delivered emergently due to Torsade's with hydrops, with ongoing arrhythmia despite medical management requiring implantation of pacemaker and sympathectomy. Early recognition of LQTS is important to optimize fetal survival with prompt medical management.

Tachy-bradycardia syndrome (TBS) has been reported rarely in the fetus. We present here an unusual dominant involvement of sinoatrial node in a fetus presenting with TBS of anti-Sjögren'syndrome-related antigen A (SS-A) and antigen B (SS-B) autoantibodies positive mother. Prenatal imaging findings, Doppler hemodynamics, and outcomes are described.

Cardiac involvement in T-cell lymphoma is not uncommon. Pericardial effusion is the most common manifestation of cardiac involvement with restrictive cardiomyopathy (RCM) due to tumor infiltration being extremely rare. The presence of paroxysmal nocturnal dyspnea and orthopnea in a patient presenting with pericardial effusion could be related to tamponade or underlying myocardial disease. Hence, reevaluation after pericardiocentesis is warranted. We describe a 14-year-old boy with advanced T-cell lymphoma presenting with cardiac tamponade. Repeat echocardiography after pericardiocentesis revealed mediastinal mass infiltrating cardiac chambers and great vessels along with features of RCM secondary to tumor infiltration.

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder.

An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51. A diagnosis of Hughes–Stovin syndrome (vascular variant of Behcet's syndrome) was confirmed, and she was started on immunosuppression, on which there was improvement.

Scimitar syndrome is a rare constellation of cardiovascular anomalies consisting of partial right anomalous pulmonary venous drainage to the inferior vena cava, hypoplasia of the right lung, cardiac dextroposition, and in many cases, one or more aortopulmonary collaterals from the descending aorta to the hypoplastic lung. The “infantile form” commonly presents with significant heart failure and pulmonary hypertension. Stenosis of the left pulmonary veins associated with scimitar syndrome is a rare but well-described combination, usually associated with a poor prognosis. We describe two patients with this association in whom the left pulmonary vein stenosis manifested months after the initial diagnosis as a progressive lesion that complicated the course of the disease due to severe pulmonary hypertension. Both patients were successfully treated with a combination of surgical, percutaneous, and hybrid treatment.

Bicuspid aortic valve (BAV) is the most common congenital cardiac defect, commonly associated with valve dysfunction and coarctation of aorta. Rarely, BAV can be associated with abnormalities of the coronary arteries, the most common of which are ectopic coronary origins. In this report, we present a case of a 2-year-old child with BAV, without coexisting supravalvular aortic stenosis, who was found to have a left main coronary ostio-proximal stenosis, leading to ischemic cardiomyopathy and congestive heart failure.

We report an 11-year-old female child presenting with hemorrhagic pericardial effusion causing cardiac tamponade along with moderate left ventricular dysfunction, who screened positive for Coxsackie B infection in the setting of cough, shortness of breath, and chest pain. She needed emergency pericardiocentesis. She also had massive bilateral hemorrhagic pleural effusions requiring bilateral chest drains placement. With a presumed diagnosis of acute myopericarditis, she was treated with steroids and ibuprofen. She made a full recovery without any further recurrence of pericardial or pleural effusion.

A 3-year-old girl presenting with fever, mucocutaneous inflammation, and acute gastrointestinal symptoms met criteria for the multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C). Echocardiography showed severely decreased left ventricular (LV) function with an apical mass. After treatment with intravenous (IV) immunoglobulin, IV steroids, anakinra, milrinone, and systemic anticoagulation, her LV function rapidly improved and the mass became increasingly mobile. Given the risk of systemic embolization, the mass was excised through left ventriculotomy and pathology confirmed a thrombus.

An aneurysm of major aortopulmonary collateral in an adult with congenital cyanotic heart disease was detected incidentally following a hemoptysis episode. The location and size of the aneurysm needed special concern during treatment to avoid aneurysm-related complications and thromboembolism secondary to intervention.

A 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.