title: Unusual clinical presentation of rare adult survivors with a missing pulmonary valve associated with a missing left pulmonary artery.
abstract: Tetralogy of Fallot with absent pulmonary valve syndrome is associated with absence of the left pulmonary artery in one-seventh of cases. Infants with this disorder commonly present very early in life with respiratory distress and heart failure. Because the pulmonary blood flow is primarily to the right lung only, severe respiratory distress occurs. Survival to late childhood without surgery is very rare due to clinical worsening in infancy. This report describes two adult natural survivors with this rare association who presented with extremely unusual unreported clinical features. The aneurysmally dilated main pulmonary artery was compressed on the left coronary ostium, causing angina in the first patient. The second patient had recurrent ventricular tachycardia originating from the dilated thinned out right ventricular outflow tract. Both patients had successful surgical correction with valved xenograft conduits.
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, MIOT Hospital, 4/112, Mount Poonamalle Road, Chennai, 600089, Tamilnadu, India, drkumarsiva@hotmail.com.
authors.forename: Kothandam
authors.initials: K
authors.lastname: Sivakumar
authors.forename: Sreeja
authors.initials: S
authors.lastname: Pavithran
authors.forename: Robert
authors.initials: R
authors.lastname: Coelho
mesh_terms: D000293:Adolescent; D000792:Angiography; D006328:Cardiac Catheterization; D003937:Diagnosis, Differential; D004452:Echocardiography; D006801:Humans; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D055815:Young Adult