Profile of Cardiac Involvement in Children After Exposure to COVID-19.

OBJECTIVE: To evaluate the incidence and pattern of cardiac involvement in children post-COVID (coronavirus disease) infection in a tertiary care referral hospital in India. METHODS: A prospective observational study was conducted including all consecutive children with suspected MIS-C referred to the cardiology services. RESULTS: Of the 111 children with mean (SD) age was 3.5 (3.6) years, 95.4% had cardiac involvement. Abnormalities detected were coronary vasculopathy, pericardial effusion, valvular regurgitation, ventricular dysfunction, diastolic flow reversal in aorta, pulmonary hypertension, bradycardia and intra-cardiac thrombus. The survival rate post treatment was 99%. Early and short-term follow-up data was available in 95% and 70%, respectively. Cardiac parameters improved in majority. CONCLUSION: Cardiac involvement post COVID-19 is often a silent entity and may be missed unless specifically evaluated for. Early echocardiography aided prompt diagnosis, triaging, and treatment, and helps in favorable outcomes.

Unexpected late-onset aortic valvulitis and moderate regurgitation during longitudinal evaluation of atypical infantile Kawasaki disease: The heart beyond coronaries!

Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve. AR without aortic root dilatation from persistent inflammation of the valve leaflets in KD is a rare phenomenon. This case demonstrates unusual cardiac manifestations of KD and reoriented our protocol for long-term surveillance in infantile KD.

Indian Academy of Pediatrics Position Paper on Kawasaki Disease.

OBJECTIVE: To formulate practice guidelines on diagnosis and management of Kawasaki disease (KD) for Indian children. JUSTIFICATION: KD is a systemic vasculitis that predominantly affects infants and children less than 5 years of age. Coronary artery abnormalities (CAA) develop in around 15-25% of untreated children with KD. Coronary artery involvement can lead to long-term cardiovascular implications such as development of premature coronary artery disease. Diagnosis of KD is essentially clinical based on recognition of a constellation of characteristic symptoms and signs. Timely diagnosis and initiation of intravenous immunoglobulin (IVIG) therapy is known to produce five-fold reduction in the incidence of CAA. As there is no confirmatory laboratory test for KD, the diagnosis may be missed if one is not familiar with the nuances of clinical diagnosis. PROCESS: A committee was formed under the auspices of Indian Academy of Pediatrics in early 2018 for preparing guidelines on KD in Indian children. A meeting of the consultative committee was held in Mumbai, and a draft protocol was devised. All members scrutinized the recent publications on the subject and an attempt was made to arrive at a broad consensus. Published guidelines on the subject were also reviewed. RECOMMENDATIONS: The diagnosis is clinical and is aided by laboratory and 2D echocardiography. First line of therapy is IVIG, and should be started expeditiously once the diagnosis is made.

Dilated superior vena cava in fetal echocardiographic three-vessel view: what lies ahead?

A 38-year-old woman, 37+3 weeker was incidentally detected to have fetal cardiomegaly during 36 weeks ultrasound and referred for fetal echocardiogram. Antenatal history and anomaly scan were normal. Fetal echocardiogram showed heart rate of 153/min, sinus rhythm, situs solitus, levocardia, dilated right atrium and ventricle with venoatrial, atrioventricular and ventriculoarterial concordances. All cardiac valves were normal; cardiac crux was intact with separate mitral and tricuspid annuli (figure 1A-C and online supplementary video 1). Mild tricuspid regurgitation with peak velocity of 1.77 m/s was noted. There was no evidence of Ebstein's anomaly. Interventricular septum was intact. Pulmonary veins were seen entering left atrium (LA) without a retrocardiac chamber. Both aortic and ductal arches were adequate but with significant flow reversal visualised in descending aorta. Three-vessel view showed dilated right superior vena cava (SVC) with normal spatial arrangement and sizes of aorta and pulmonary artery. Patent foramen ovale and patent ductus arteriosus were shunting normally. Inferior vena cava (IVC), hepatic and portal veins were normal. Significant hepatomegaly was seen without any evidence of hydrops. 10.1136/heartasia-2018-010998.supp1Supplementary data Figure 1(A) Fetal echocardiogram showing atrioventricular concordance with right atrium (RA) connected to morphological right ventricle and left atrium (LA) connected to morphological left ventricle (LV). Blue arrow depicts a large foramen ovale shunting from RA to LA. There is no retrocardiac chamber behind LA. (B) Three-vessel view in fetal echocardiogram showing normal right to left arrangement of superior vena cava (SVC), aorta (Ao) and pulmonary artery (PA). Interestingly, SVC is bigger than its neighbours which is against the norm. (C) Fetal short-axis echocardiographic view showing situs solitus with normal arrangement of RA and LA. RA is draining into right ventricle which is draining through PA into the ductal arch. However, significant aortic run-off is noted in aorta. QUESTION: As per the available echocardiographic data, what is the most likely diagnosis for fetal heart failure?Idiopathic dilatation of SVC?Supracardiac total anomalous pulmonary venous connection?Fetal vein of Galen malformation?Fetal anaemia?

Catastrophic presentation of anomalous origin of right pulmonary artery in a neonate--an interesting differential!

A 36-hour-old term neonate presented with refractory cardiogenic shock and multi-organ dysfunction. On suspecting coarctation of aorta, intravenous prostaglandin was started but without any benefit. Echocardiography revealed isolated anomalous origin of right pulmonary artery from ascending aorta causing massive aortic steal, supra-systemic pulmonary arterial hypertension (PAH), multi-organ dysfunction and right ventricular failure. The main pulmonary artery was solely continued as left pulmonary artery. Emergency surgery at 48 h was life-saving and mid-term follow-up is excellent. The authors present this case as an important alternative diagnosis to duct dependent systemic lesions. The English literature in the last two decades was reviewed to discuss the characteristics, the diagnosis and treatment of neonatal presentation of this rare anomaly. Incidentally, the reported case is the youngest successful survivor of this surgery.

Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome.

We present a 3-year-old child with Budd Chiari syndrome having idiopathic complete occlusion of all three major hepatic veins and small hepatic venules. Adequate antegrade flow in right hepatic vein was established by transfemoral balloon angioplasty followed by stenting of the same. Long term antiplatelet therapy was instituted. Medium term follow up reveals satisfactory antegrade flow and regression of symptoms. This strategy highlights an effective nonsurgical approach of restoring physiological pattern of hepatic sinusoidal blood flow in Budd Chiari syndrome.

Percutaneous device closure of a bifurcated inferior vena cava and completion of the Kawashima procedure for the "difficult" univentricular heart.

Azygos continuation of inferior venacava (IVC) is well known in association with interrupted IVC. We report azygos connection of uninterrupted IVC in a young child with complex univentricular heart. This peculiar anatomy was made suitable for univentricular repair by combining percutaneous device closure of prehepatic right limb of IVC and bidirectional Glenn shunt. The azygos vein acted as a conduit for IVC flow to superior venacava as in Kawashima's operation. This case highlights a rare IVC morphology. Also this innovative strategy allowed simple solution to a challenging problem and is first time described in the literature.