Efficacy, safety and tolerability of bosentan as an adjuvant to sildenafil and sildenafil alone in persistant pulmonary hypertension of newborn (PPHN).

Background: Pulmonary Arterial Hypertension (PAH) carries a poor prognosis in both adult and pediatric patients. It is a life-threatening condition in newborns. Current recommendations advocate the use of targeted monotherapy as a first-line approach for the treatment of Persistent Pulmonary Hypertension of the Newborn (PPHN). In case of an inadequate clinical response to treatment, an addition of a second or third agent is considered. PAH is usually managed with a phosphodiesterase 5 inhibitor or an endothelin receptor blocker. There are limited pediatric studies that address questions like which class of therapy should be initiated first or if a combination should be initiated together. With this background, the present study was initiated to compare the efficacy, safety, and tolerability of bosentan as an adjuvant to sildenafil and sildenafil alone in PPHN. Results: A total of 40 patients were enrolled in the study. Out of them, 26 were males (65%) and 14 were females (35%). PPHN was most commonly seen in the 29 (72.5%) of participants with a history of first order birth. Mean duration of symptoms was 14.05 ± 2.06 days. The participants were randomized to two groups. Group A consisted of total 25 participants that received both bosentan and sildenafil and group B had 15 participants that received sildenafil alone. Both groups were comparable in terms of birth weight and present weight, consanguinity, and mode of delivery. Efficacy was determined by the reduction in mean baseline Pulmonary Artery Systolic Pressure (PASP). PASP in group A was 75.56 ± 10.62 mm Hg and in group B was 64.86 ± 12.25 mm Hg which was not statistically significant (P > 0.05). PASP on the third and seventh day in group A were 43.72 ± 8.63 and 24.47 ± 3.52 mm Hg compared to 42.28 ± 9.43 and 27.276 ± 8.38 respectively in group B which was statistically significant (P < 0.05).There were two deaths each in both groups. Two participants in Group A developed liver function abnormalities. None of the participants in Group B had adverse effects. Conclusion: Most common clinical manifestations were nonspecific. Cardiovocal syndrome was common in PPHN. We conclude that oral sildenafil treatment is a safe, simple and effective treatment for persistent pulmonary hypertension in newborn. Combination of bosentan with sildenafil is more effective and safe in reducing pulmonary artery (PA) pressures in high-risk patients with PPHN.

Unguarded left atrioventricular orifice: An unusual cause of hypoplastic left ventricle and double-outlet right ventricle with intact ventricular septum.

An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care.

Three-dimensional transesophageal echocardiography-guided transcathetar closure of ruptured noncoronary sinus of valsalva aneurysm.

Sinus of Valsalva aneurysm accounts for only 1% of congenital cardiac anomalies. Sinus of Valsalva aneurysm can cause aortic insufficiency, coronary artery flow compromise, cardiac arrhythmia, or aneurysm rupture. Three-dimensional transesophageal echocardiography (3DTEE) represents an adjunctive tool to demonstrate the ruptured sinus of Valsalva with better delineation. We present an adult patient with rupture of noncoronary sinus of Valsalva aneurysm into the right atrium (RA). 3DTEE accurately delineated the site of rupture into the RA and showed the exact size and shape of the defect, which helped in the successful transcatheter closure of the defect with a duct occluder device.

Clinical, angiographic profile and percutaneous endovascular management of Takayasu's arteritis - A single centre experience.

OBJECTIVE: Aim of the study was to evaluate clinical, angiographic profile and percutaneous endovascular management of Takayasu's arteritis. BACKGROUND: Takayasu's arteritis is a chronic inflammatory vasculitis affecting the aorta and its major branches. Although it is more prevalent in Asia, the distribution of the disease is worldwide with different vascular involvement patterns and clinical manifestations. METHODS: In this prospective study a total of 50 consecutive patients who were reported as having Takayasu's arteritis between January 2010 and April 2016 were evaluated. Detailed clinical presentation and angiograms of all patients were analysed. RESULTS: 50 patients were analysed during study period. Among 50 patients, 43(86%) were female and 7 (14%) were male. Average age of presentation was 26.92years. Most common clinical presentation was claudication (74%) followed by, musculoskeletal symptoms (48%), fatigue (46%), weight loss (22%), headache (22%), visual disturbances (16%), syncope (10%), dyspnoea (20%). Most common features were absent/diminished pulses (80%), difference in blood pressure (80%), followed by bruit (70%)hypertension (64%), cerebrovascular accident (8%),heart failure (8%) and aortic regurgitation (4%). According to the new angiographic classification, angiographic type I (40%) was encountered most frequently, followed by type III (30%), type V (16%), type IIb (8%), type IIa (2%), and type IV is (4%). Angioplasty was the main stay of treatment in 66% of the patients, remaining 34% of them were treated medically either with corticosteroids or methotrexate. CONCLUSION: Takayasu's arteritis is a rare disease, affects mainly women, manifestations range from asymptomatic disease, found as a result of impalpable pulses or bruits, to catastrophic neurological impairment. Takayasu's arteritis is the common cause of renovascular hypertension. Angiography remains the gold standard for diagnosis. Angiographic evaluation and percutaneous transluminal angioplasty with stenting is useful in selected cases.

Ebstein's anomaly with subpulmonary obstruction--a rare association.

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3-year-old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.

Percutaneous transcatheter device closure of coronary arteriovenous fistula through persistent left superior vena cava.

Congenital coronary artery fistula is a rare malformation that may involve any or all coronary artery branches and any cardiac chamber. Elective closure of coronary artery fistula by surgery or percutaneous transcatheter technique is generally accepted in the presence of symptoms. Controversies exist in the management of asymptomatic patients. We report a case of successful percutaneous transcatheter device closure of an aneurysmally dilated coronary fistula, originating from the circumflex artery and draining into the coronary sinus, through the persistent left superior vena cava. Persistent left superior vena cava facilitated the easier closure of the fistula, with short procedural and fluoroscopy times.

Retrograde approach for closure of ruptured sinus of Valsalva.

Though ruptured sinuses of Valsalva have been traditionally managed surgically, they are amenable to transcatheter closure. Various devices have been used for closure of these defects. We describe a novel technique of closure of a ruptured right sinus of Valsalva into the right ventricular outflow tract. A muscular ventricular septal defect occluder was deployed retrogradely, without resorting to the usual antegrade technique involving formation of an arteriovenous loop.