[Total anomalous drainage of the pulmonary veins-- surgical therapy for the infradiaphragmatic and mixed anatomical types].
OBJECTIVE
To study the in-hospital evolution of patients with the infradiaphragmatic and mixed types of total anomalous pulmonary venous drainage (TAPVD), who underwent surgical therapy.


METHODS
Of the 65 patients diagnosed with isolated TAPVD and operated on from December 1993 to March 2002, 7 (10.8%) patients with the mixed and infradiaphragmatic forms were retrospectively selected. Their ages ranged from 5 days to 19 months (mean of 7 months), 5 (71.4%) were males, and their clinical diagnosis was established with 2-dimensional echocardiography. Four (57.1%) patients had the mixed form, which was intrinsic obstructive in 1 patient, with mild stenosis of the left inferior vein. The remaining 3 (42.9%) patients had the obstructive infradiaphragmatic form, extrinsic at the level of the diaphragm. All surgeries were performed through median sternotomy with hypothermic extracorporeal circulation, and total circulatory arrest was required in 2 patients.


RESULTS
In-hospital death occurred in 1 patient with infradiaphragmatic TAPVD with connection of the inferior vertical vein with the portal vein. The cause of death was related to multisystem organ failure. In 4 (57.1%) patients, the postoperative period was characterized by the presence of low cardiac output and pulmonary hypertension.


CONCLUSIONS
The result of the surgical correction of this anomaly is associated with acceptable morbidity and mortality, depending on early referral and surgery, without progression of the pulmonary vascular hypertension findings.
Atik
Fernando Antibas
FA
Instituto do Coração, Hospital das Clínicas, FM, USP. atik@uol.com.br
Irun
Patricia Egusquiza
PE
Barbero-Marcial
Miguel
M
Atik
Edmar
E
D003964:Diaphragm / Q000002:abnormalities / Q000601:surgery; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014655:Vascular Resistance