Partial anomalous right pulmonary venous connection to inferior vena cava. Clinical and surgical experience

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Abstract

We analize the clinical and surgical course of 18 patients who have partial anomalous right pulmonary venous connection to inferior vein cava as a part of scimitar syndrome (SC: dextrocardia, right lung hypoplasia, pulmonary sequestration). They were divided into: Group I (pulmonary systolic pressure ~. 40 mmHg), composed by 12 patients with mean age of 7.8 years; and Group II (pulmonary systolic pressure ~ 41 mmHg), with 6 patients and mean age 0.10 years old. All were submitted to hemodynamic and angiographic evaluation. The Group I was asymptomatic; in 10 patients the surgical approach was performed without early or late mortality. The Group II, with critical clinical status in the newborn period, had pulmonary hypertension and had 33 % of medical- surgical mortality. The cardiac catheterization is a definitive and essential study for understand- ing the physiopathological ground regarding to select the optimal therapeutical management. Surgical closure or transluminal embolization of the systemic arteries is the first stage proposed in infants and neonates; the complete reconnection of venous channel to left atrium will be deferred as a second stage. In children and adults the last surgical method is indicated as a function of pulmonary blood flow.