title: Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy.
abstract: 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.
authors: Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Medical College P O, Thiruvananthapuram, 695011, India, raghu_rak@hotmail.com.
authors: Krishnan A
authors: KA
authors: Raghuram
authors: Sasidharan
authors: S
authors: Bijulal
authors: Kavasseri M
authors: KM
authors: Krishnamoorthy
authors: Jaganmohan A
authors: JA
authors: Tharakan
mesh_terms: D000015:Abnormalities, Multiple; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods*; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D006330:Heart Defects, Congenital / Q000628:therapy*; D059446:Heterotaxy Syndrome / Q000175:diagnosis*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology; D007223:Infant; D008297:Male; D010879:Piperazines / Q000627:therapeutic use*; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use*; D014655:Vascular Resistance / Q000502:physiology*; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate