內臟轉錯症隨伴 左心症 : 合倂 大血管轉錯症에 對한 手術 1例 Levocardia with Situs Inversus Viscerum:A complicated combination of anomalies = Levocardia with complete transposition of the greate vessels and pulmonary stenosis:A case of successful palliative surgery

다국어 초록 (Multilingual Abstract)

A six years old Korean girl was admitted in February 1963, with cyanotic congenital heart disease.
By auscultation grade I -Ⅱ systolic murmur was audible along the left sternal border without thrill.
Remarkable laboratory findings were high degree of and hematuria, polycythemia, albuminuria, and right sided hemiparesis.
Transposition of the abdominal viscera with levocardia was found on routine X-ray study
ECG showed right axis deviation and inverted P-wave in lead I .
The right heart catheterization and angiocardiography with Shonander serial X-ray machine showed the right-sided (venous)atrium receiving the right and left superior vena cava separately, complete transposition of the great vessels, shunt at the ventricular level, and pulmonary stenosis.
The inferior vena cava coursed along the left border of the spinal column and drained into the venous atrium, which is in the left hemithorax.
The aortic arch was left-sided and bifurcated right ureter was visualized. Glenn' s procedure was done to increase the pulmonary blood flow in March 1963.
Postoperative course was benign and uneventful.
The follow-up study of the case, five month postively, revealed satisfactory results; increased exercise tolerance, marked relief from dyspnea on exertion, abolished cyanosis on rest, incresed arterial oxygen saturation (from 48% to 70%), normalization of hemogram, and increased pulmonary vascularity on the right lung field.
The final anatomical findings of this unusually complicated cardiac anomaly were:
(1) Levocardia with situs inversus viscerum, chamber inversion, and inverted P-wave in Lead I on ECG.
(2) Transposition of the great vessels with pulmonary stenosis.
(3) Intra-cardiac shunt or Shunts:
i. Undetermined intracardiac shunt, Vntricular septal defect or single vent -ricle or atrioventricularis communis ?
ii. Confirmed atrial septal defect.
(4) Double superior vena cavae: left superior vena cava drained into the right atrial cavity (right atrium proper or coronary sinus?).
Inferior vena cava on the left side. (By course of the cardiac catheter) Left aortic arch and left descending aorta.
Bifurcated right ureter.
Asplenia :presence not determined.
Three lobes of the right lung confirmed by operation but numbr of lobes on the left lung undetermined.
This report is believed to be the first operative palliation case of complete trans-position of the great vessels with pulmonary stenosis in a Korean literature.