Unexpected complication of uncovered aortic end in ductal stenting.

A newborn with tricuspid atresia and pulmonary atresia underwent ductal stenting. The aortic end of the ductus was not completely covered and was wide open; the baby was discharged on dual antiplatelets. The baby presented after a month with desaturation, and an angiogram showed extensive thrombus in the stent and the right pulmonary artery. The angle formed by the stent with the uncovered aortic end is likely to have precipitated the thrombus.

Cervical origin of left subclavian artery: A rare anomaly.

A 22-year-old lady was referred to our institute for the management of pulmonary atresia with hypoplastic pulmonary arteries. Computed tomographic Angiography (CTA) showed right aortic arch with left brachicephalic artery as the first branch, which trifurcated into internal carotid, external carotid and subclavian artery high up in the neck at the level of third cervical vertebra. The left subclavian artery then travelled back caudally and entered into the arm after giving rise to a large collateral artery. This is the first ever-reported case of cervical origin of left subclavian artery (COLSA) in the literature. This anomaly can be explained by the absence of left fourth aortic arch with left subclavian artery arising from the left third aortic arch.

An unusual cause for unruptured sinus of valsalva aneurysm.

Sinus of valsalva aneurysm is considered to be one of the rarest complications of inflammatory aortitis. Herewith, we are reporting a young male patient who presented to us with severe aortic regurgitation. On evaluation, he was found to have unruptured sinus of valsalva aneurysm. CT angiography and magnetic resonance imaging have shown value in the diagnosis of sinus of valsalva aneurysm.

Association between gender, process of care measures, and outcomes in ACS in India: results from the detection and management of coronary heart disease (DEMAT) registry.

BACKGROUND: Studies from high-income countries have shown that women receive less aggressive diagnostics and treatment than men in acute coronary syndromes (ACS), though their short-term mortality does not appear to differ from men. Data on gender differences in ACS presentation, management, and outcomes are sparse in India. METHODS AND RESULTS: The Detection and Management of Coronary Heart Disease (DEMAT) Registry collected data from 1,565 suspected ACS patients (334 women; 1,231 men) from ten tertiary care centers throughout India between 2007-2008. We evaluated gender differences in presentation, in-hospital and discharge management, and 30-day death and major adverse cardiovascular event (MACE; death, re-hospitalization, and cardiac arrest) rates. Women were less likely to present with STEMI than men (38% vs. 55%, p<0.001). Overall inpatient diagnostics and treatment patterns were similar between men and women after adjustment for potential confounders. Optimal discharge management with aspirin, clopidogrel, beta-blockers, and statin therapy was lower for women than men, (58% vs. 65%, p = 0.03), but these differences were attenuated after adjustment (OR = 0.86 (0.62, 1.19)). Neither the outcome of 30-day mortality (OR = 1.40 (0.62, 3.16)) nor MACE (OR = 1.00 (0.67, 1.48)) differed significantly between men and women after adjustment. CONCLUSIONS: ACS in-hospital management, discharge management, and 30-day outcomes did not significantly differ between genders in the DEMAT registry, though consistently higher treatment rates and lower event rates in men compared to women were seen. These findings underscore the importance of further investigation of gender differences in cardiovascular care in India.

Percutaneous transjugular device closure of postoperative residual atrial septal defect.

Percutaneous closure of postoperative malaligned residual atrial septal defect was successfully performed from the transjugular approach under transesophageal echocardiography guidance in a 38-year-old symptomatic woman with patent femoral venous access using the usual hardware. This demonstrates the feasibility of transjugular approach as an alternative to femoral or transhepatic approaches in patients with difficult atrial septal anatomy who are usually referred for surgery.

Percutaneous closure of a moderate to large tubular or elongated patent ductus arteriosus in children younger than 3 years: is the ADO II appropriate?

Protrusion of the Amplatzer duct occluder (ADO) II device into the aortic isthmus or the pulmonary artery causing obstruction and residual flow has been reported, but the same has not been widely studied in small children with a patent ductus arteriosus (PDA) anatomy not considered suitable for closure with the ADO I device. This study aimed to report the safety and efficacy of the ADO II device in children younger than 3 years with a tubular or elongated PDA and to analyze the possible reasons for residual flow in children with such a PDA. In this study, 17 children younger than 3 years (mean age, 10.3 ± 7 months; mean weight, 6 ± 3.6 kg) underwent attempted closure of a tubular or elongated PDA (mean diameter at the narrowest point, 4.1 ± 1.1 mm) with the ADO II device between July 2010 and July 2012. Of the 17 patients, 16 (2 boys and 14 girls) completed the follow-up evaluation. A complete echocardiographic evaluation was performed on all the patients before PDA closure and at the follow-up visit, and the results were compared with those of previous published studies. Of the 16 patients, the 15 who completed the follow-up evaluation had successful device closure (1 device embolization). Residual flow was present in six patients immediately after deployment, which was reduced to three patients at the last follow-up visit. Five of nine patients closed with a 6-mm-long device had residual flow compared with only one of seven patients closed with a 4-mm-long device. After device closure, significant elevations of the left and right pulmonary artery velocities occurred in three and two patients, respectively; in 12 patients, descending thoracic aortic (DTA) velocities increased mildly. There was trend toward a fall in the elevated pressures at the last follow-up visit, although one patient had an elevation in right pulmonary artery velocity at last the follow-up echocardiogram compared with the echocardiogram immediately after closure. Hence, in children younger than 3 years with or without pulmonary arterial hypertension, closure of a PDA not amenable to closure with the ADO I device is feasible using the ADO II device, with an increased incidence of clinically nonsignificant complications. Selection of device dimensions according to the manufacturer's recommendation may not be the optimal strategy.

Unusual association of aortic valve stenosis with ventricular septal defect and pulmonary atresia: Differentiation from truncus arteriosus with truncal valve stenosis.

Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.

Differential diagnosis of vascular structures in relation to upper ascending aorta: The retro-aortic innominate vein.

The retroaortic course of left innominate vein is a rare entity which can be misinterpreted during echocardiography for other abnormal vascular structures under the arch of aorta. We report the case of a 2 month old infant where the suprasternal window showed 2 vascular structures beneath the aortic arch, one of which was traced to be a retroaortic innominate vein.

Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy.

A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.

Septal course of left anterior descending artery from the right aortic sinus in tetralogy of Fallot: a benign anomaly and important lessons learned.

The proximal course of an anomalously arising coronary artery is a decisive factor in the surgical approach for tetralogy of Fallot (TOF). Studies have shown that echocardiography provides a good anatomic definition of the ostium and proximal epicardial course of coronary arteries [1, 2]. This report describes a case of TOF with an atrioventricular canal defect whose preoperative echocardiography showed abnormal origin of the left anterior descending artery (LAD) from right aortic sinus, which was interpreted as crossing the right ventricular outflow tract. Perioperative inspection did not show any abnormal vessel crossing the outflow, and corrective surgery was performed. At the echocardiographic evaluation after surgery, it was noted that the abnormal LAD arising from right aortic sinus was taking a septal course in relation to the posterior aspect of the pulmonary annulus. It is important to recognize this anomalous course because it is benign with no surgical implications.

Crisscross pulmonary artery with double aortic arch: an unusual association.

Anomalies of pulmonary artery origin are rare. Crisscross pulmonary artery origin is a rare benign anomaly characterized by the left pulmonary artery arising superiorly and to the right side of the right pulmonary artery. The condition is usually accompanied by a conotruncal anomaly. Here, we report a child with crisscross pulmonary arteries and a complete vascular ring formed by a double aortic arch, which was confirmed by computed tomography angiography. The child underwent surgical correction for relief of stridor.

Kodamaea ohmeri tricuspid valve endocarditis with right ventricular inflow obstruction in a neonate with structurally normal heart.

The yeast Kodamaea (Pichia) ohmeri is a rare human pathogen with infrequent report of neonatal infection. Native valve endocarditis by Kodamaea ohmeri is extremely rare. The current case report describes a case of fatal nosocomial native valve endocarditis without any structural heart defects in a 40dayold baby. The patient was referred to our institute after having ICU stay of 18 days in another hospital for necrotizing enterocolitis and was found to have obstructive tricuspid valve mass and fungemia with Kodamaea ohmeri. In spite of the treatment, patient developed sepsis with disseminated intravascular coagulation and could not be revived.

Anomalous pulmonary venous connection to superior vena cava: Warden technique.

OBJECTIVE: Repair of anomalous pulmonary venous connection (APVC) to superior vena cava (SVC) with internal pericardial patch may be complicated by the obstruction of the SVC or pulmonary veins and sinus node dysfunction. Moreover, the shrinkage of the pericardium may lead to late obstruction. Various techniques are practiced, which have reduced the incidence of obstruction to the venous drainage, but sinus node dysfunction continues to be an issue. We reviewed our experience with the Warden technique, predominantly in children, for various types of APVC to the SVC. METHODS: Between 2006 and 2010, 32 patients with anomalous drainage of the pulmonary veins to SVC underwent repair by the Warden technique in our institute. The median age at operation was 4 years (range 3 months-34 years). Partial anomalous pulmonary venous connection (PAPVC) was present in 28 patients, while four patients had total anomalous pulmonary venous connection (TAPVC) to the SVC, where one or more pulmonary veins joined the SVC separately from the entry of the common chamber. Clinical data, echocardiography and operative details were collected from our database, retrospectively. RESULTS: At a median follow-up of 24 months, there was no mortality. One patient had transient rhythm disturbance. On follow-up, it was revealed that all patients are in sinus rhythm with no evidence of systemic or pulmonary venous obstruction. CONCLUSION: The Warden technique is a simple and effective surgical option, which should be preferred for patients with anomalous drainage of the pulmonary veins to SVC. Though arrhythmias are rare in the early follow-up, longer follow-up is required to rule out their late development.

Late presentation of aortic root abscess in endocarditis with coronary ischemia.

Formation of a large aortic root abscess is an infrequent complication of aortic valve endocarditis in adults. Extrinsic compression of the coronary arteries by this abscess is still rarer. Here, we report a case of a 22-year-old male with aortic root abscess, who presented 2 months after the completion of treatment of endocarditis with exertional angina. Coronary angiogram revealed compression of proximal left anterior descending and left circumflex arteries by the abscess. The patient was successfully treated with pericardial patch exclusion of the abscess cavity and coronary artery bypass graft. The presentation of aortic root abscess with myocardial ischemia as a late complication of treated endocarditis has not been reported earlier.

Natural history of aortic valve disease following intervention for rheumatic mitral valve disease.

BACKGROUND AND AIM OF THE STUDY: A significant proportion of patients who require interventions for rheumatic mitral valve (MV) disease have coexisting aortic valve (AV) disease. To date, little is known of the natural history of AV disease in these patients. METHODS: The details of a cohort of 200 patients (146 females, 54 males; mean age at MV intervention 30.3 +/- 9.9 years) with rheumatic heart disease were retrospectively reviewed. The patients had undergone an index MV intervention (either closed or balloon mitral valvotomy) or MV replacement between 1994 and 1996, and received long-term regular follow up examinations. The clinical and echocardiographic data at entry and at follow up were noted. Patients were allocated to two groups, based on whether the AV disease was absent (group I, n=98) or present (group II, n=102) at baseline. The AV disease was categorized as thickening only (group IIA), isolated aortic regurgitation (AR) (group IIB), or combined aortic stenosis (AS) and AR (group IIC). No patient had isolated AS at baseline. RESULTS: The mean follow up period was 9.3 +/- 1.07 years; during which 10 patients in group I developed new AV disease, which included AV thickening only (n=2), trivial-mild AR (n=7) and mild AS with trivial AR (n=1). Of 16 patients in group IIA, 11 developed isolated AR, and one patient progressed to have mild AS and AR. Among 69 patients in group IIB, 22 (31.9%) developed AS, and all had either mild (n=8) or moderate (n=14) AR with mild AS. Group IIC included 17 patients with mild combined AV disease at baseline, except for moderate AS and moderate AR in one patient each. Among 16 patients with mild AS in group IIC, six progressed to moderate AS and two to severe AS. AR became moderate in 10 patients and severe in one patient. The two patients who progressed to severe AS requiring AV replacement had mild AS at baseline. No patient who developed new combined AV disease had lesions with severity more than mild AS or moderate AR. On logistic regression analysis of the variables predisposing to progression of AV disease, such as age, gender, history of rheumatic fever (RF) and recurrence, and interval from RF episode to symptom onset, only the initial AV gradient was identified as being statistically significant (beta coefficient 0.528, SE = 0.17, p < 0.0001). CONCLUSION: Patients with no or mild AV disease at the time of MV intervention rarely develop severe AV disease, and seldom require AV surgery over the long-term follow up. The presence of mild AS at baseline is predictive in the minority of cases where AV disease will progress relatively more rapidly.

An unusual thrombotic complication during percutaneous closure of atrial septal defect.

A case is described where intracardiac thrombus was observed at the junction of the superior vena cava and the right atrium during attempted closure of an atrial septal defect (ASD) using a device similar in design to the Amplatzer septal occluder (Heart R atrial septal occluder). Thrombosis was likely related to the multiple attempts required when deploying the device and the attendant prolonged procedural time. The patient underwent subsequent urgent surgical closure of the ASD.