Aortico right atrial tunnel - Clinical presentation, transcatheter management, and follow-up from a large cohort of patients.

Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients. Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis. Results: Among 21 patients including 8 males with a median age of 3 years (18 days-72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing <10 kg presented early with heart failure. Transcatheter closure normalized the hemodynamics including in one infant after failed surgery. Two elderly patients (10%) above 60 years presented with angina and atrial fibrillation. The rest were asymptomatic. Occluders were positioned in the narrow proximal aortic end of the tunnel in all except two patients, where the distal atrial end was closed. All procedures were successful without complications. There was one late death after 1 year from subarachnoid hemorrhage. At a median follow-up of 96 months, all survivors were asymptomatic. Thromboprophylaxis with dual antiplatelets for 1-2 years followed earlier was recently changed to aspirin with Coumadin. Complete remodeling occurred when the proximal aortic end was closed, but partial persistence of the track was noted after distal closure. Conclusions: This largest cohort of ARAT showed the safety and efficacy of transcatheter closure even in neonates. The narrow proximal aortic end should be the target for closure rather than the distal atrial end to achieve complete remodeling.

Unexpected stormy course after uneventful device closure of atrial septal defect - Possibly due to post-COVID-19 inflammatory state.

COVID-19 infection has myriad manifestations from self-limiting illness to stormy multi-organ failure. A 28-year-old woman negative for COVID reverse transcription-polymerase chain reaction underwent an uneventful elective device closure of atrial septal defect on intubation anesthesia. While a brief postprocedural endotracheal bleed was noted, significant hypoxia and respiratory distress ensued after extubation with biventricular dysfunction, pleural effusion, and radiographic evidence of acute respiratory distress syndrome. COVID antibodies were positive, and inflammatory markers were elevated. After a conservative multipronged medical management including anticoagulation, antibiotics, aspirin, beta-blocker, diuretics, and sildenafil, she improved in 1 week. The clinical course during this pandemic era gives a possibility of a post-COVID inflammatory syndrome as a potential etiology.

Late occurrence of ventricular septal rupture after deep septal myectomy for hypertrophic cardiomyopathy: Causes and management.

Surgical septal myectomy is increasingly utilized for patients with hypertrophic obstructive cardiomyopathy who remain symptomatic despite maximum doses of medical therapy. Deep and extensive septal muscle resections may lead to iatrogenic ventricular septal defects that are detected on transesophageal echocardiography immediately after weaning from cardiopulmonary bypass and immediately corrected in the same surgery. However markedly thinned out ventricular septum after myectomy may be prone to late rupture from high left ventricular systolic pressures causing delayed detection of a ventricular septal defect when the patients present with new onset symptoms. Additionally, a surgical injury to the first septal perforator artery during the myocardial resection leading to septal infarction may contribute to delayed occurrence of ventricular septal defect. Such a predisposing deep septal resection or septal infarction may be associated with varying degrees of atrioventricular nodal block warranting a permanent pacing. A new onset interventricular shunt from such an iatrogenic ventricular septal defect often leads to heart failure as the filling pressures increase disproportionately in the thick hypertrophied left ventricle. Transcatheter closure is an alternative to a high-risk repeat surgery. This report of device closure of two delayed septal ruptures after myectomy discusses the reasons, presentation, catheter approaches, and procedural challenges. .

Cardiac Evaluation in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated With COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) is notorious for its cardiac involvement. We present a single center data of 71 children, of which 57.7% had myocarditis and 26.8% had coronary artery aneurysms. 45.1% required intensive care support and 29.6% needed inotropes - 91.5% received IVIG.  All patients responded to therapy with no mortality.

Simultaneous balloon aortic valvotomy and patent ductus arteriosus device closure in preterm newborn.

Multiple congenital heart disease in the small preterm newborn such as severe narrowing of aortic valve and patent ductus arteriosus (PDA) is a therapeutic challenge. We report successful transcatheter antegrade balloon dilatation of the aortic valve and device closure of the PDA in a 1700-gram preterm newborn. Meticulous planning and team work aids in such transcatheter intervention. .

Cardiac catheterization through ipsilateral radial and ulnar artery access during the same procedure.

We evaluated the safety and feasibility of ipsilateral radial and ulnar artery cannulation during the same catheterization procedure. Crossover from radial to femoral was done in 122 patients. Both ipsilateral radial and ulnar catheterization were performed in 16 patients without any complications, which was further supported by Doppler ultrasonography.

Echocardiographic Classification of Perimembranous Ventricular Septal Defect Guides Selection of the Occluder Design for Their Transcatheter Device Closure.

BACKGROUND: Perimembranous ventricular septal defects (VSDs) has proximate relation to the aortic and tricuspid valves as well as the conduction tissues. Transcatheter closure utilizes various off-label device designs. METHODS: Perimembranous VSD without aortic margin were classified as group A, with thick aortic margin as group B, with membranous septal aneurysm as group C and defects restricted by tricuspid valve attachments as group D. The proposed ideal design was asymmetric device in group A; duct occluder I (ADOI) and muscular ventricular septal occluder (MVSO) in group B; thin profile duct occluder II (ADOII) in group C and ADOI in group D. Device was 0-2 mm larger than the defect. RESULTS: Eighty patients with VSD measuring 6.83 ± 2.87mm underwent successful closure. Device was retrieved before release in one group A and one group C patient due to aortic regurgitation. Asymmetric device was used in 16 group A defects. Among group B defects, ADOI was used in 5, ADOII in 5, MVSO in one and asymmetric device in 3. Group C defects were closed with ADOI in 7, ADOII in 10 and asymmetric device in 3. Three patients with multiple exits had 2 ADOII devices. Group D defects were closed using ADOI in 20 and ADOII in 10 patients. There was no late aortic regurgitation or heart block on a follow-up exceeding 7 years. CONCLUSIONS: This echocardiographic classification helps device selection in every single patient. While asymmetric device is uniquely suited for group A defects, different designs are appropriate in the other groups.

Radiofrequency ablation for fascicular ventricular tachycardia causing tachycardiomyopathy and brief literature review.

A 10-years-old boy presented with a history of effort intolerance and palpitations for 4 months. His electrocardiogram showed wide complex tachycardia suggestive of fascicular ventricular tachycardia (VT). The echocardiogram showed moderate-to-severe left ventricular systolic dysfunction without any structural lesion. The tachycardia was unresponsive to adenosine and direct current cardioversion. It responded to oral verapamil. The electrophysiology study confirmed the tachycardia as left posterior fascicular VT. The tachycardia was successfully ablated guided by Purkinje potential on three-dimensional mappings. He showed improvement in ventricular functions before discharge. He is doing well on short-term follow-up.

Percutaneous closure of large pulmonary artery to left atrial fistula.

Pulmonary artery to the left atrial fistula is a rare anomaly. Two young males presented with effort intolerance and cyanosis and had large pulmonary artery to left atrial fistula from right and left pulmonary artery, respectively. The right-sided fistula was closed with a large duct occluder device in transseptal approach whereas the left-sided fistula was closed with a large muscular ventricular septal device. Complete occlusion and symptomatic relief was achieved in both cases. In the follow up the patients were doing well.

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.

Anomalous origin of right coronary artery causing myocardial ischemia in a young patient.

Anomalous right coronary artery from left coronary sinus can have dynamic narrowing and kinking causing symptoms of myocardial ischemia and sudden cardiac death. Surgical repair of the anomaly is required in the symptomatic patient because of risk of ischemia or ventricular arrhythmia. Asymptomatic incidentally diagnosed low-risk patients can be closely followed up with exercise restriction as per present guideline.

A rare case of cardiac tumor in a child.

Pediatric cardiac tumors are rare and usually benign. An infectious etiology like tuberculosis invading myocardium and presenting as infiltrative mass is extremely rare. We present a case of a 15 month old girl with clinical feature of cardiac failure who had infiltrative multiple myocardial masses in echocardiogram. Advanced cardiac imaging by Cardiac Magnetic resonance imaging (MRI) helped in tissue delineation. Therapeutic trial of anti-tubercular drugs in view clinical suspicion of Tuberculosis resulted in complete remission of symptom and disappearance of the cardiac mass.

Juxtaposed atrial appendages: A curiosity with some clinical relevance.

If the atrial appendages lie adjacent to each other on same side of the great arteries, instead of encircling their roots, they are referred as juxtaposed. Right juxtaposition of atrial appendages is less common than left juxtaposition. The images demonstrate the classical radiological, echocardiographic, and surgical images of juxtaposed atrial appendages. Their clinical incidence, associations, and relevance during interventional and surgical procedures are discussed.

A novel method of creation of a fenestration in nitinol occluder devices used in closure of hypertensive patent arterial ducts.

Test occlusion with a balloon is done to predict operability of large hypertensive patent ductus arteriosus (PDA). If the fall in the pulmonary artery pressures is inadequate, a complete closure is not desired. To create a predictable premeasured fenestration in a nitinol occluder device used for closing hypertensive PDA. A large nitinol occluder device was punctured with an 18G needle to advance a 0.035˝ stiff guide wire through the occluder before loading it into the delivery system. The occluder with the guidewire was then deployed across the PDA. A coronary guide catheter was later threaded through the guidewire into the fabric of the device, which was still held by the delivery cable. A coronary stent was deployed across the fenestration in the occluder to keep it patent. An 8-year-old boy with Down syndrome and hypertensive PDA was hemodynamically assessed. Even though there was a fall in the pulmonary vascular resistance index and pressures on test occlusion, the pulmonary artery pressures were labile with fluctuations. A customized fenestration was made in a 16 mm muscular ventricular septal defect occluder (MVSO) with a 4.5 mm bare-metal coronary stent. The pulmonary artery pressures remained at half of the aortic pressures after the procedure. This fenestration model precisely and predictably fenestrated a large occluder device used to close a hypertensive large PDA. Long-term patency of these fenestrations has to be assessed on the follow-up, and may be improved through larger fenestrations, systemic anticoagulation and use of covered stents.

Plastic bronchitis.

Plastic bronchitis, a rare but serious clinical condition, commonly seen after Fontan surgeries in children, may be a manifestation of suboptimal adaptation to the cavopulmonary circulation with unfavorable hemodynamics. They are ominous with poor prognosis. Sometimes, infection or airway reactivity may provoke cast bronchitis as a two-step insult on a vulnerable vascular bed. In such instances, aggressive management leads to longer survival. This report of cast bronchitis discusses its current understanding.

Evaluation of Congenital Valvular Heart Diseases by the Pediatrician: When to Follow, When to Refer for Intervention?

Isolated congenital valvular heart disease in children constitutes a small fraction of congenital heart diseases. Valve involvement is seen more along with other congenital diseases. The most commonly involved valve is the pulmonary valve followed by the aortic valve. Stenotic lesions of the pulmonary and aortic valves are more frequently encountered than mitral and tricuspid valvular lesions. The presentation depends on the severity of the lesion and the age of the patient. Symptoms range from asymptomatic status to florid symptoms of valve obstruction and/or leak. Detailed clinical assessment and various imaging techniques confirm the diagnosis and help in management planning. Transcatheter balloon dilatation for obstructive pulmonary valve has very good long-term outcomes. The results of balloon dilation of aortic valve are also good enough for it to be the treatment of choice. Significant lesions of the mitral and tricuspid valve, regurgitant lesions, sub and supra valvular obstructions require surgical correction. Most valvar lesions mandate regular follow up. Communication and coordination between the pediatric cardiologist and the pediatrician helps in the optimal management.