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.

A rare case of type X dual left anterior descending coronary artery.

A dual left anterior descending (LAD) coronary artery is a rare, and benign congenital anomaly. In this anomaly, there is the presence of two LADs in the anterior inter ventricular sulcus (AIVS). One of the LADs is a short one that ends high in the AIVS. The other longer one enters the distal AIVS and feeds the apex. To date, 9 types of dual LAD variants, and one novel type X has been reported. Herein, we report a case of type X dual LAD with a literature review. .

Aortoiliac reconstruction in the setting of in-stent restenosis.

Complex iliac artery obstructions, particularly bilateral stenosis or total iliac artery occlusions, are usually treated with aortofemoral or aortobifemoral graft surgery. However, surgical treatment is associated with 3% mortality rate and significant morbidity such as intestinal ischemia, spinal cord injury, and ureteral damage. Percutaneous interventions of aortic bifurcation offer a promising alternative to surgery with potentially lower morbidity and mortality risk. We report a case of peripheral artery disease who had underwent right transfemoral iliac angioplasty with femoropopliteal bypass presented again with bilateral lower limb ischemia, who was successfully treated with stent implantation with the kissing balloon technique.

Amplatzer duct occluder II for closure of congenital Gerbode defects.

BACKGROUND: Congenital left ventricle to right atrial communications (Gerbode defects) are extremely rare (0.08%) type of ventricular septal defects. They were traditionally closed by surgery in the past. There are few case reports and small series of acquired and congenital Gerbode defects, closed with various types of devices. Aim of our study is to assess the feasibility, efficacy, and complications of transcatheter closure of congenital Gerbode defects with Amplatzer duct occluder II (ADO II). MATERIAL: Twelve consecutive cases of Gerbode defects, age ranging from 10 months to 16 years (mean 6.7 years), weight ranging from 6.5 kg to 34 kg (mean 19.3 kg), were diagnosed on transthoracic echocardiography. RESULTS: Transcatheter closure of Gerbode defects was done successfully through retrograde approach with ADO II. No aortic or tricuspid regurgitation or residual shunt occurred in any of the patients. One patient developed transient complete heart block needing temporary pacing. DISCUSSION: The soft low profile, easily trackable ADO II appears to be ideal for closure of Gerbode defects, as the central cylinder fits in the defect and the soft retention discs on either side, without polyester material, do not impinge on either aortic, mitral, or tricuspid valve. We report the successful transcatheter closure of twelve cases of congenital Gerbode defects with ADO II. CONCLUSIONS: Transcatheter closure of congenital Gerbode defects with ADO II is safe, effective, and an attractive alternative to surgical closure. ADO II appears to be tailor made for Gerbode defects, as the success rate is very high and complication rate is very low.