Unveiling the complexity: coexistence of rheumatic heart disease and pulmonary arteriovenous malformation-a unique case report.

Background: The coexistence of rheumatic heart disease (RHD) and pulmonary arteriovenous malformation (PAVM) is a rare clinical scenario that poses diagnostic and therapeutic challenges. This case report explores the clinical presentation, diagnostic journey, and multidisciplinary management of a patient presenting with both conditions. Case summary: A 47-year-old female with a history of RHD presented with symptoms of dyspnoea on exertion and cyanosis, suggestive of both cardiac involvement and pulmonary involvement. Subsequent investigations involving imaging, echocardiography, and invasive pulmonary angiography revealed the coexistence of RHD and multiple PAVM in the patient's left lower lobe of the lung. The patient underwent a tailored treatment plan, initially involving percutaneous mitral balloon valvuloplasty for RHD, followed by a staged procedure of transcatheter PAVM closure with Amplatzer™ Vascular Plug II performed 1 month later. Her saturation normalized following the intervention. The patient's progress was monitored closely, with adjustments made to the treatment plan based on evolving clinical scenarios. The patient remained well in short-term follow-up. Discussion: This case highlights the complexity of managing patients having two diverse conditions RHD and PAVM coexisting together, thus emphasizing the importance of a multidisciplinary approach. The unique intersection of cardiac and pulmonary pathologies necessitates careful consideration of diagnostic nuances and tailored treatment strategies. Lessons learned from this case offer valuable insights for clinicians encountering similar scenarios and underscore the significance of individualized, patient-centred care in optimizing outcomes for those with dual pathologies.

Transcatheter Device Closure of Perimembranous Ventricular Septal Defect in Pediatric Patients: Long-Term Outcomes.

AIMS: The aim of this study is to evaluate the safety and efficacy of transcatheter device closure of perimembranous ventricular septal defects in pediatric patients at long-term follow-up. MATERIALS AND METHODS: We prospectively studied 376 patients with perimembranous VSDs between September 2008 and December 2015 who underwent percutaneous closure at our center. Transthoracic echocardiography (TTE) and electrocardiogram were done before and after the procedure in all the patients. All patients were subjected to follow-up evaluation at 1, 3, 6, 12 months, and annually thereafter with TTE and electrocardiogram. RESULTS: A total of 376 patients (210 males and 166 females) underwent transcatheter closure of perimembranous VSD. Mean age of patients was 8.67 ± 3.02 (range 3-18 years) and mean weight was 21.15 ± 8.31 (range 8-65 kg). The procedure was carried out successfully in 98.93% of patients with no reported mortality. Rhythm disturbances occurred in 8.5% of patients after the procedure which included three cases of complete atrioventricular block. CONCLUSION: This study shows that in experienced hands transcatheter closure of perimembrnous VSD is safe and effective at long-term follow-up. With minimal morbidity and no mortality, the transcatheter is an effective alternative to surgical closure in selected patients.

Long-Term Results of Transcatheter Closure of Large Patent Ductus Arteriosus with Severe Pulmonary Arterial Hypertension in Pediatric Patients.

AIMS: Patent ductus arteriosus (PDA) is one of the most commonly seen congenital heart diseases prevalent today. The aim of this study is to evaluate the safety and efficacy of transcatheter closure of hypertensive ductus at long-term follow-up. MATERIALS AND METHODS: Transcatheter closure was attempted in 52 patients with hypertensive ductus arteriosus. A lateral or right anterior oblique view aortogram was done to locate and delineate PDA. All the patients underwent clinical examination, electrocardiography, chest X-rays, and echocardiography before discharge and at 1, 6, and 12 months after the procedure and yearly thereafter. RESULTS: The mean age of patients at procedure was 7.98 ± 4.79 (11 months-17 years), and the mean weight was 17.72 ± 10.81 (4-47) kg. Transcatheter closure of hypertensive ductus was successful in 50 (96.15%) patients. The mean preprocedural pulmonary artery pressure was 81.38 ± 17.31 (range: 55-113) mmHg which decreased to 29.65±8.63 (19-38) mmHg at follow up. The most commonly used device was Amplatzer duct occluder in 63% of the patients followed by Amplatzer muscular ventricular septal defect occluder in 37% of the patients. There were two procedural failures, namely aortic obstruction and left pulmonary artery stenosis, which were managed uneventfully. There were no procedural deaths or device embolization. At median follow-up of 86 months, all the patients are well with no complications. CONCLUSION: The long-term results suggested that transcatheter closure of PDA with severe pulmonary hypertension in pediatric patients is safe and effective with minimal complications.

Diagnostic Accuracy of Computed Tomography Angiography as Compared to Conventional Angiography in Patients Undergoing Noncoronary Cardiac Surgery.

OBJECTIVE: To compare the diagnostic accuracy of multi-slice computed tomography (MSCT) angiography with conventional angiography in patients undergoing major noncoronary cardiac surgeries. MATERIALS AND METHODS: We studied fifty major noncoronary cardiac surgery patients scheduled for invasive coronary angiography, 29 (58%) female and 21 (42%) male. Inclusion criteria of the study were age of the patients ≥40 years, having low or intermediate probability of coronary artery disease (CAD), left ventricular ejection fraction (LVEF) >35%, and patient giving informed consent for undergoing MSCT and conventional coronary angiography. The patients with LVEF <35%, high pretest probability of CAD, and hemodynamically unstable were excluded from the study. RESULTS: The diagnostic accuracy of CT coronary angiography was evaluated regarding true positive, true negative values. The overall sensitivity and specificity of CT angiography technique was 100% (95% confidence interval [CI]: 39.76%-100%) and 91.30% (95% CI: 79.21%-97.58%). The positive (50%; 95% CI: 15.70%-84.30%) and negative predictive values (100%; 95% CI: 91.59%-100%) of CT angiography were also fairly high in these patients. CONCLUSION: Our study suggests that this non-invasive technique may improve perioperative risk stratification in patients undegoing non-cardiac surgery.

Transcatheter device occlusion of a large pulmonary arteriovenous fistula by exit closure: the road less travelled.

Large pulmonary arteriovenous fistula (PAVF) manifests as cyanosis and predisposes to serious complications of right-to-left shunt, and therefore necessitates early treatment. The emergence of antegrade transcatheter closure of feeding arteries as treatment of choice is limited by inherent risk of either recanalization or reappearance of new feeders and potential risk of systemic embolization. Additional closure of the draining vessel by transcatheter device occlusion should overcome the limitations of conventional antegrade technique. We describe two cases of successful transcatheter closure of a large PAVF by antegrade device closure of feeders as well as transseptal retrograde closure of the exiting channel.

Clinical profile of pediatric patients with long QT syndrome masquerading as seizures.

OBJECTIVE: To study the clinical profile of patients with long QT syndrome presenting as seizures. METHODS: Retrospective analysis of six pediatric patients admitted at authors' institute between October 2008 and January 2013 with seizures and a presumptive diagnosis of long QT syndrome (LQTS) was done. The diagnosis was made on the basis of updated Schwartz diagnostic criteria. Clinical data, investigation profile and follow up of patients was recorded in a standard format and analysed. RESULTS: All the 6 patients in the study were boys with a mean age of 10.3 ± 2.8 y at the time of diagnosis. The lag period between symptom onset and diagnosis was 5.6 ± 3.14 y. All patients had history of seizures with a history of precipitating event in 4 patients. Average baseline QTc interval was 556 ± 41.31 ms. Mean Schwartz score was 6.66 ± 1.16. Polymorphic VT was documented in 4 patients. After initiating standard treatment with betablockers, nicorandil, spironolactone or pacemaker, all the six patients were asymptomatic at a mean follow up period of 17.5 mo, with no recurrence of seizures. CONCLUSIONS: LQTS can cause seizures due to prolonged ventricular arrhythmias in high risk subgroup. Children, who present with LQTS and seizures, generally have a precipitating event causing seizures, and they respond well to drug therapy.