Significance of pulmonary venous obstruction in total anomalous pulmonary venous return.
BACKGROUND
Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis. It has been detected more frequently in recent year due to the advent of echocardiography and cardiovascular magnetic resonance imaging (MRI). The aim of this study was to evaluate the clinical manifestations and outcomes in TAPVR patients with or without pulmonary venous obstruction (PVO).


METHODS
From January 1985 to December 2002, a total of 27 cases with TAPVR at our institution were reviewed. Accurding to the preseace or assence of PVO, patients were divided into PVO group and non-PVO group. Patients' sex, age at diagnosis, types of TAPVR, clinical manifestations, surgical treatment and outcomes were evaluated.


RESULTS
All of them had received 2-dimensional (2-D) and color Doppler echocardiography examination. Cardiac catheterization was performed in all but 1 patient who died at the first day of birth. In addition, 10 of 27 cases had cardiovascular MRI for further study. The number of cases in PVO group and non-PVO group were 15 (56%) and 12 (44%), respectively. There was no significant difference in sex or pulmonary venous drainage sites between both groups. Cyanosis was more prevalent in the PVO group (80% vs. 30%, p = 0.038). Four (27%) cases PVO group and 3 (25%) cases of the non-PVO group had of the non-isolated cardiac lesions. Pulmonary hypertension was present in 18 (69%) of 26 cases who had received cardiac catheterization. Among them, 10 had PVO and 5 had systemic level of pulmonary arterial pressure. Seven (30%) of 23 patients who had received operation died; in contrast, 3 of 4 patients without operation expired. The remaining 1 did not had surgery because of complex heart disease. There was no significant difference in surgical mortality between PVO and non-PVO groups (33% vs. 27%).


CONCLUSIONS
Cyanosis is an obvious clinical symptom of obstructed TAPVR. Surgical mortality made no significant difference between obstructed and non-obstructed groups. Early detection and surgical treatment for TAPVR are important. Although cardiac catheterization and angiocardiography is the golden standard for the diagnosis, 2-D and color Doppler echocardiography can also provide quick and accurate diagnostic images of TAPVR.
Wang
Ping-Yao
PY
I-Lan Hospital, Department of Health, Taipei, Taiwan, ROC.
Hwang
Be-Tau
BT
Lu
Jen-Her
JH
Lee
Pi-Chung
PC
Tiu
Chui-Mei
CM
Weng
Zeng-Chung
ZC
Meng
Luara C C
LC
D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D006769:Hospitals, General; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011667:Pulmonary Veins / Q000473:pathology / Q000503:physiopathology*; D011668:Pulmonary Veno-Occlusive Disease / Q000473:pathology / Q000503:physiopathology*; D012189:Retrospective Studies; D013624:Taiwan; D014694:Ventricular Outflow Obstruction / Q000473:pathology / Q000503:physiopathology*