sildenafil-citrate and Abnormalities--Multiple

Tetralogy of Fallot (TOF) with pulmonary atresia (PA) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of congenital heart disease with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in pulmonary hypertension (PH). To date, reports regarding the role of PH medications in this population are sparse. The objective of this study was to assess the effect of PH medications in patients with TOF, PA and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for patients at a single tertiary care pediatric center. Twelve of 66 patients were treated with PH medications (18 %), and eight of these patients had adequate follow-up for further analysis. Median age at last follow-up was 6 years (range 1.4-21 years). Median length of therapy with PH medication was 4 years (range 0.3-17 years). PH medications included sildenafil, bosentan, ambrisentan, inhaled treprostinil and prostacyclin infusion. PH therapy was associated with improvement in symptoms in all patients and improvement in PH by hemodynamic measures in the majority of patients. All patients underwent at least one cardiac intervention by catheterization or surgery while taking PH medication. Two patients died from non-PH-related causes. The remaining six patients are alive and remain on PH medication. This review indicates that PH medications are well tolerated by this patient group and provide symptomatic improvement. Further studies are required to determine whether PH medications provide long-term survival benefit for patients with complex congenital heart disease.

Topics: Abnormalities, Multiple; Adolescent; Antihypertensive Agents; Bosentan; Catheterization; Child; Child, Preschool; District of Columbia; Echocardiography; Female; Hemodynamics; Hospitals, Pediatric; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Lung; Male; Phenylpropionates; Pulmonary Artery; Pulmonary Atresia; Pyridazines; Retrospective Studies; Sildenafil Citrate; Sulfonamides; Tertiary Care Centers; Tetralogy of Fallot; Young Adult

We report a 14-month-old child with persistent truncus arteriosus and crossed pulmonary arteries. The potential advantage of crossed pulmonary artery arrangement in achieving surgical correction is discussed.

Topics: Abnormalities, Multiple; Anastomosis, Surgical; Cardiomegaly; Cardiopulmonary Bypass; Combined Modality Therapy; Dobutamine; Female; Humans; Hypertension, Pulmonary; Infant; Milrinone; Pulmonary Artery; Sildenafil Citrate; Truncus Arteriosus, Persistent; Vascular Surgical Procedures

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.

Topics: Abnormalities, Multiple; Cardiac Catheterization; Cardiac Surgical Procedures; Disease Progression; Familial Primary Pulmonary Hypertension; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Hypertension, Pulmonary; Infant; Male; Piperazines; Purines; Sildenafil Citrate; Sulfones; Vascular Resistance; Vasodilator Agents

Topics: Abnormalities, Multiple; Arteriovenous Fistula; Cardiac Catheterization; Child, Preschool; Endothelium-Dependent Relaxing Factors; Female; Follow-Up Studies; Heart Bypass, Right; Heart Defects, Congenital; Humans; Hypoxia; Nitric Oxide; Piperazines; Postoperative Complications; Pulmonary Artery; Purines; Radiography; Risk Assessment; Sildenafil Citrate; Sulfones; Treatment Outcome; Vascular Resistance; Vasodilator Agents