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[Successful primary correction for tetralogy of Fallot associated with total anomalous pulmonary venous drainage
M Shinonaga1, S Eguchi, H Miyamura
1Second Department of Surgery, Niigata University School of Medicine, Japan.
Insights
Successful surgical correction for a child with Tetralogy of Fallot and total anomalous pulmonary venous drainage demonstrates primary repair is viable, even with pulmonary hypertension and small left ventricle.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
- Surgical Innovation
Background:
- Tetralogy of Fallot (TOF) with total anomalous pulmonary venous drainage (TAPVD) is a complex congenital heart defect.
- Preoperative assessment revealed significant pulmonary hypertension and a small left ventricle, posing surgical challenges.
Observation:
- A 3-year-old girl underwent successful surgical correction for combined TOF and TAPVD (Type Ia).
- Postoperative circulatory instability was noted, requiring close monitoring.
- Two-dimensional echocardiography tracked left ventricular (LV) volume changes during recovery.
Findings:
- LV volume significantly increased by postoperative day 8, coinciding with circulatory stabilization.
- Pre-discharge cardiac catheterization showed normalized LV volume and acceptable pulmonary-to-systemic flow ratio (Pp/Ps of 0.38).
Implications:
- Primary surgical repair is feasible and effective for this complex cardiac anomaly in pediatric patients.
- Early intervention can lead to favorable long-term outcomes despite initial adverse hemodynamic parameters.
- This case supports surgical correction even in the presence of preoperative pulmonary hypertension and reduced LV volume.
Abstract:
The surgical correction was performed successfully in a 3-year-old girl with tetralogy of Fallot associated with total anomalous pulmonary venous drainage (type Ia). The preoperative cardiac catheterization and angiography showed high systolic pulmonary artery pressure (58 mmHg) and small left ventricular volume (54% of normal). The early postoperative course was stormy because of the unstable circulatory state. During the early postoperative period, we estimated the volume of left ventricle by 2 D echocardiography. A significant increase of the volume was observed on the 8th postoperative day, when her circulatory state became stable. The cardiac catheterization before discharge revealed Pp/Ps of 0.38 and normal LV volume. We conclude that primary repair should be done for this combined cardiac anomaly, even if small left ventricle and pulmonary hypertension is present.