Postnatal Left Ventricular Outflow Tract Obstruction Caused by Multiple Rhabdomyoma Tumors / 대한흉부외과학회지

Rhabdomyoma is the most common benign cardiac neoplasm in neonates. Most patients with rhabdomyoma experienced spontaneous regression. Yet some of them need surgical therapy because of hemodynamic problems of the heart such as arrhythmia, outflow tract obstruction and valvular dysfunction. We found multiple masses in both ventricles on the patient's fetal echocardiogram. Heart failure caused by severe left ventricular outflow tract obstruction quickly presented after birth. The mass interfering with the outflow tract was resected via the transaortic approach at the first day of birth. Postoperative echocardiography showed complete release of the outflow tract obstruction. He was discharged on the postoperative day 8. During the 3 years of follow up, we found that the sizes of the remnant masses had gradually decreased.

Left Ventricular Outflow Tract Obstruction Caused by Accessory Mitral Valve Tissue in a Child: A case report / 대한흉부외과학회지

Accessory mitral valve tissue is a very rare congenital cardiac malformation and it is an uncommon cause of left Ventricular outflow tract obstruction (LVOTO). The pathogenetic mechanism of subaortic obstruction is thought to be systolic ballooning of the abnormal valve tissue into LVOT. We are reporting a case of an accessory mitral valve tissue that was associated with LVOTO, and this was completely relieved after trans-aortic surgical excision of the accessory tissue.

The Clinical Application and Results of Palliative Damus-Kaye-Stansel Procedure / 대한흉부외과학회지

BACKGROUND: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. MATERIAL AND METHOD: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months (13 days~38.1 months) and body weight was 5.0 kg (2.9~13.5 kg). Preoperative pressure gradients were 25.3+/-15.7 mmHg (10~60 mmHg). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6, another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. RESULT: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of 62.7+/-38.9 months (3.3~128.1 months). Kaplan-Meier estimated actuarial survival was 71.9%+/-9.3% at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. CONCLUSION: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

The Norwood-Rastelli Procedure for Left Ventricular Outflow Tarct Obstruction with a Ventricular Septal Defect: Three case report / 대한흉부외과학회지

Between 2001 and 2006, 3 neonates that had multilevel left ventricular outflow tract obstruction and a ventricular septal defect underwent the Norwood-Rastelli procedure. The body weights ranged from 2.9 to 3.1 kg. The patients had a near normal sized mitral valve and left ventricle. We simultaneously performed a modified Norwood procedure with native tissues-to-tissue anastomosis without circulatory arrest, and a Rastelli type procedure using a non-valved conduit from the right ventricle to the pulmonary artery and intracardiac patch baffling from the left ventricle to the pulmonary valve via the ventricular septal defect. The postoperative courses were uneventful. During follow-up, there was one late mortality caused by a cardiac catheterization related complication at 7 months after surgery. One patient required a Rastelli conduit change. Two patients are doing well during a follow-up period of 1 and 5 years, respectively.

A Case Report of Restrictive Ventricular Septal Defect & Left Ventricular Out Flow Tract Stenosis Caused by Fibrotic Tissue in Tetralogy of Fallot / 대한흉부외과학회지

The VSD in TOF is usually large and unrestrictive with an equal to or greater than that of the aortic annulus. Typically shunting through the VSD is bidirectional or right-to-left component. Restrictive VSD in TOF caused by ingrowing fibrotic tissue is very rare. We report a case of restrictive VSD and LVOTO in TOF caused by ingrowing fibrotic tissue with the review of literature.

Clinical Analysis of Surgical Results for Discrete Subaortic Stenosis / 대한흉부외과학회지

BACKGROUND: Discrete subaortic stenosis is known to recur frequently even after surgical resection. We retrospectively reviewed the preoperative and postoperative changes in pressure gradient through left ventricular outflow tract, and the recurrence rate. MATERIAL AND METHOD: Between September 1984 and December 2004, 34 patients underwent surgical treatment. Mean age of patients was 17.1+/-15.2 years and 19 patients (55.9%) were male. 16 patients (47.1%) had previous operations and associated diseases were aortic regurgitation (11), coarctation of aorta (3), and others. RESULT: Immediate postoperative peak pressure gradient was significantly lower than preoperative peak pressure gradient (21.8 mmHg vs 75.8 mmHg, p<0.01). Peak pressure gradient measured after 50.3 months of follow up was 20.2 mmHg, which was also significantly lower than that of preoperative value but not significantly different from that of immediate postoperative value. There was no surgical mortality but one patient developed cerebral infarction. Mean follow up duration was 69.8+/-54.6 months. During this period, 5 patients (14.7%) had reoperation, 3 (8.8%) of whom were due to recurred subaortic stenosis. We found no risk factors for recurrence and survival for free from reoperation was 76.4%. CONCLUSION: Excision of subaortic membrane combined with or without myectomy in discrete subaortic stenosis showed sufficient relief of left ventricular outflow tract obstruction with low mortality and morbidity, but careful long term follow up is necessary for recurrence, since it is not predictable.

Surgical Results of Complete Atrioventricular Septal Defect: 16 years experience / 대한흉부외과학회지

BACKGROUND: In this study, we reviewed our early and long-term surgical results of complete atrioventricular septal defect during the last 16 years at our hospital. MATERIALS AND METHODS: Between April 1986 and March 2002, 73 patients with complete atrioventricular septal defect underwent total correction without preceding palliation. Age at repair ranged from 2 to 85 (median age, 7) months, and weight ranged from 3 to 22 (median weight, 5.9)kg. Follow-up was complete with a mean duration of 69+/-51 months. RESULTS: Overall operative mortality was 16.4%(12) with 3 late deaths. One, 5, and 10 year actuarial survival rates were 96.3%, 94.2%, and 94.2% respectively. Sixteen of 61 (22.2%) operative survivors have undergone reoperation for postoperative mitral regurgitation or left ventricular outflow tract obstruction (LVOTO). Freedom from mitral reoperation at 1, 5, and 10 years were 87.8%, 72.4%, and 57.8% and freedom from LVOTO at 1, 5, and 10 years were 98.2%, 86.3%, and 83.2% respectively. SUMMARY: In this study, we found that our early surgical results improved with quite an acceptable long-term outcome. Close observation of remaining mitral regurgitation was necessary. A precise evaluation of the atrioventricular valve morphology, a meticulous surgical technique, and the adequate postoperative management are mandatory for the excellent results.

Rapid Left ventricular Training after Arterial Switch Operation in Transposition of Great Arteries with Left Ventricular Outflow Tract Obstruction and ventricular Septal Defect: 1 case report / 대한흉부외과학회지

There have been few reports documenting the outcome of arterial swich operations(ASO) in selected patients with transposition of great arteries(TGA) and with left ventricular outflow tract obstruction(LVOTO). In the case of TGA with LVOTO, if the atrial septal defect(ASD) is large and the ventricular septal defect(VSD) is restricive, this deprives the left ventricle(LV) of approporiate preload and could lead to underdevelopment of the ventircular mass and lead poor LV performance after the arterial switch operation, dspite a high pressure in the LV preoperatively. Because an increase in the systolic ventricular pressure is not necessarily paralleled by an increase in ventricular mass, which is also essential for optimal ventricular performance after the operation. We report here a case of rapid LV training after ASO in TGA with unprepared LV (because of large ASD and restrictive VSD) despite a high pressure in the LV(due to LVOTO) preoperatively.

Successful Removal of Left Ventricular Rhabdomyoma : A Rare Cause of Left Ventricular Outflow Obstruction in the Newborn Infant: 1 case report / 대한흉부외과학회지

Cardiac rhabdomyomas are the most common primary tumor in infancy and childhood and are frequently associated with tuberous sclerosis. Although spontaneous regression of cardiac rhabdomyoma has been reported , prognosis of this tumor associated with subaortic stenosis is still considered to be poor and surgery continues to be indicated. A 4-day-old female was admitted due to tachypnea and cyanosis. Single rhabdomyoma arising from the interventricular septum associated with severe subaortic stenosis was partially removed under cardiopulmonary bypass. Excised tumor size was 0.7 X 0.9 X 0.4 cm in dimension. The postoperative course was uneventful and the infant discharged on the 14th postoperative day.

Successful Removal of Left Ventricular Rhabdomyoma : A Rare Cause of Left Ventricular Outflow Obstruction in the Newborn Infant: 1 case report / 대한흉부외과학회지

Cardiac rhabdomyomas are the most common primary tumor in infancy and childhood and are frequently associated with tuberous sclerosis. Although spontaneous regression of cardiac rhabdomyoma has been reported , prognosis of this tumor associated with subaortic stenosis is still considered to be poor and surgery continues to be indicated. A 4-day-old female was admitted due to tachypnea and cyanosis. Single rhabdomyoma arising from the interventricular septum associated with severe subaortic stenosis was partially removed under cardiopulmonary bypass. Excised tumor size was 0.7 X 0.9 X 0.4 cm in dimension. The postoperative course was uneventful and the infant discharged on the 14th postoperative day.