The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.
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 ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). 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 $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ 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.
Background: Sinus valsalva aneurysm (SVA) is a rare disease, and it is frequently accompanied by ventricular septal defect and aortic valve regurgitaion. For treatment of SVA, several surgical mordalities were applied, but there was no report on the long-term result after surgical repair in Korea. We reviewed our 28 years of experiences and analyzed the long-term results after treatment of sinus valsalva aneurysm with or without rupture. Material and Method: Between March 1974 and February 2002, 81 patients were operated under the impression of sinus valvsalva aneurysm or sinus valsalva aneurym rupture. Retrospectively we reviewed the patients' record. Mean age of patients was $29.2\pm11.5$ and there were 49 males. Accompanyng diseases were as follows: VSD in 50, PDA in 2, Behcet's disease in 2, TOF in 1, RVOTO in 1, AAE in 1. Seventy-seven $(95\%)$ patients had sinus valsalva rupture and in 14 patients, subacute bacterial endocarditis was accompanied. Degree of aortic valve regurgitation was as follows: grade I: 8, II: 10, III: 9, IV: 4. Most common rupture site was right coronary sinus (66 patients, $81\%$) and most common communication site was right ventricle (53 patients). In repair of sinus valsalva rupture, patch was used in 37 patients, and direct suture was done in 38 patients. Result: There was one surgical death $(1.2\%)$. Follow up was done in 78 patients $(97.5\%)$, mean follow up period was $123.3\pm80.9(3\~330\;months)$. During the follow up period, 3 patients died $(3.8\%)$. One patient died of heart failure, another patient died of arrhythmia and the other one died of unknown cause. In two patients, complete atrio-ventricular block was developed during follow up period, and there was no operation related event or complication. Kaplan-Meier survival analysis revealed $92.5\pm3.5\%$ survival at 15 and 27 years and it seems to be satisfactory. Conclusion: Long-term surgical results and survival is satisfactory after repair of sinus valsalva aneurysm with or without rupture.
Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.
Aneurysm of the sinus of Valsalva is fairly rare. Between 1987 and 1994, we operated on a total of 12 cases of aneurysm of the sinus of Valsalva at the Inje University Seoul Paik Hospital. This represents 0.7% of all cardiac operation under cardiopulmonary bypass. There were eight male and four female patients aged from 8 to 38 years(mean, 19.2 years). 8 of 12 patients had ruptured aneurysms. The origin of aneurysm of the sinus of Valsalva was the right coronary sinus in 10(83. 3%), and the noncoronary sinus in 2(16.6%). In ruptured aneurysm, the origin was the right oronary sinus in 6, and the noncoronary sinus in 2. The aneurysms originating from the right coronary sinus ruptured into the right ventricle in 5, and into both the right atrium and right ventricle in 1. The aneurysms originating from the noncoronary sinus ruptured into the right ventricle in 1, and into the right atrium in 1. Associated congenital cardiac defects included ventricular septal defect in 10(83.3%) patients 39 cases of these were associated with the aneurysms of the right coronary sinus), aortic regurgi- tation in 3 (all of these had an additional ventricular septal defect), mitral regurgitation in 1, and double chambered right ventricle in 1. No hospital deaths occurred, although one late death occurred as a result of endocarditis 15 months after the first operation. The mean follow-up period was 29 months, range from 4 to 60 months. Eleven patients except one late death were in New York Heart Association class 1. Due to the low mortality risk o( an operation for aneur sm of the sinus of Valsalva, a ruptured aneurysm of the sinus of Valsalva should be corrected surgically when the diagnosis is mane, and unruptured aneurysm of the sinus of Valsava with complication should also be operated. In most cases the aorta was opened to examine the morphology of the aneurysm and the aortic cusps, and an associated aortic valve defect should be corrected simultaneously.
We investigated changes of the size of neoaortic annulus, root, and aortic anastomosis after arterial switch operation for complete transposition of the great arteries performed in infancy. A total of 23 patients were included in this study. Age ranged from 6 to 153 days. Body weight averaged 3.9$\pm$0.8kg and 17 patients were male. The preoperative angiocardiographic dimensions of the pulmonary annulus, the pulmonary root, and the sinotubular junction, standardized to the diameter of descending aorta at the level of diaphragm, were compared to the size of postoperative measurements of the neoaortic annulus, the neoaortic root, and the aortic an stomosis at a mean interval of 17.2$\pm$ 9.4 months. Mean dimensions of the neoaortic annulus and the neoaortic root were significantly increased postoperatively(n=23, annulus; p<0.01, root; p<0.01), however, those of the aortic anastomosis did not reveal significant change(n=23, p=0.06). There were no significant differences in changes of diameters of the neoaortic annulus, the root, and the aortic anastomosis between patients with(n=8) and without(n=15) postoperative neoaortic regurgitation(annulus; p=0.32, root; p=0.29, anastomosis; p=0.86). Postoperative dimensions of the neoaortic root and annulus between patients with ventricular septal defect(n: 10) and without ventricular septal defect(ni 13) were not significantly changed compared to the preoperative measurements(annulus; p=0.09, root; p=0.07) but mean diameters of the aortic anastomosis decreased significantly after operation in patients with ventricular septal defect(p=0.04). This study revealed that the site of the aortic an stomosis grows in proportion to patient's somatic growth after arterial switch operation. Although we could not demonstrate the relation between the aortic root dilatation and the postoperative neoaortic regurgitation in this study, a continuous close follow-up might be necessary to detect a possible progression of the aortic root dilatation and the resulting significant aortic valve regurgitation.
Background: Infective endocarditis shows high surgical mortality and morbidity rates, especially for aortic endocarditis. This study attempts to investigate the clinical characteristics and operative results of isolated aortic endocarditis. Material and Method: From July 1990 to May 2005, 25 patients with isolated aortic endocarditis (Group I, male female=18 : 7, mean age $43.2{\pm}18.6$ years) and 23 patients with isolated mitral endocarditis (Group II, male female=10 : 13, mean age $43.2{\pm}17.1$ years) underwent surgical treatment in our hospital. All the patients had native endocarditis and 7 patients showed a bicuspid aortic valve in Group I. Two patients had prosthetic valve endocarditis and one patients developed mitral endocarditis after a mitral valvuloplasty in Group II. Positive blood cultures were obtained from 11 (44.0%) patients in Group I, and 10 (43.3%) patients in Group II, The pre-operative left ventricular ejection fraction for each group was $60.8{\pm}8.7%$ and $62.1{\pm}8.1%$ (p=0.945), respectively. There was moderate to severe aortic regurgitation in 18 patients and vegetations were detected in 17 patients in Group I. There was moderate to severe mitral regurgitation in 19 patients and vegetations were found in 18 patients in Group II. One patient had a ventricular septal defect and another patient underwent a Maze operation with microwaves due to atrial fibrillation. We performed echocardiography before discharge and each year during follow-up. The mean follow-up period was $37.2{\pm}23.5$ (range $9{\sim}123$) months. Result: Postoperative complications included three cases of low cardiac output in Group I and one case each of re-surgery because of bleeding and low cardiac output in Group II. One patient died from an intra-cranial hemorrhage on the first day after surgery in Group I, but there were no early deaths in Group II. The 1, 3-, and 5-year valve related event free rates were 92.0%, 88.0%, and 88.0% for Group I patients, and 91.3%, 76.0%, and 76.0% for Group II patients, respectively. The 1, 3-, and 5-year survival rates were 96.0%, 96.0%, and 96.0% for Group I patients, and foo%, 84.9%, and 84.9% for Group II patients, respectively. Conclusion: Acceptable surgical results and mid-term clinical results for aortic endocarditis were seen.
Background: Anatomic correction of transposition of the great arteries by means of the arterial switch operation is now accepted as the therapeutic method of choice. This retrospective study attempts to assess the results of the neonatal arterial switch operation for transposition of the great arteries performed by our newly established institution. Materials and methods: 33 consecutive neonates underwent the arterial switch operation between October 1991 to November 1997. There were 27 neonates with transposition and intact ventricular septum, 3 with ventricular septal defect, and 3 with Taussig-Bing anomaly. The mean age was 10.9$\pm$7.9 days and mean body weight was 3.29$\pm$0.44kg. Results: Overall postoperative hospital mortality was 30.3% (10 patients). The mortality has improved with time; 75% (6 patients) among first 8 consecutive patients before 1994, 20% (2 patients) among 10 patients in 1994 and 1995, and 13.3% (2 patients) among 15 patients since 1996. Univariated analysis of risk factors revealed that earlier date of the operations and one of preoperative events were determinants for operative death. There were two late deaths. A mean follow-up of 17.4$\pm$16.5 months was achieved in all 21 survivors. All were in New York Heart Association functional class I. One patient had mild pulmonary stenosis and two had mild aortic valve regurgitation on their echocardiography. Conclusions: We concluded that we should continue to perform arterial switch operation for neonates with transposition of the great arteries because the mortality of the operation has been improved and the operative survivors have good functional results with low incidence of late complications.
In recent years, the use of allograft conduits in repair of congenital cardiac disease is widely accepted. However, the supply of homograft. is currently limiting their increased clinical application, especially small cryopreserved homografts for use in neonates and inf'ants. We used a technique to surgically reduce the size of the more readily available large-diameter allografts, making them suitable for right ventricular outflow tract reconstruction in small infants and children. From December 1994 to March 1996, a total of 11 patients ranging in age from 10 months to 6 years (mean age, 27.3 months) and ranging in weight from 5.6 to 18.5 kg (mean 11.5 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique (pulmo ary atresia with ventricular septal defect, 9 cases ; tetralogy of Falloff, 2 cases). The diameter after downsizing ranged from 14 to 19 mm with a mean of 16.8 mm. There was one operative death due to rupture of the infected homograft. Evaluation of these patients between 2 and 15 months (mean 6.9 months) after homograft implantation reveals excellent clinical and echocardiographic results. There were no significant homograft insufficiency and RVOT obstructions. Although a longer follow-up is certainly required to evaluate the long term fate of the surgically modified bicuspid homografts, we believe that this technique may represent a valuable therapeutic alternative, at least in the short term, to the use of synthetic grafts when an appropriately sized homograft is not available.
A 12-year-old Standard Poodle presented with intermittent weakness and occasional dyspnea at the Veterinary Medicine Teaching Hospital of Kangwon National University. A grade of 4 out of 6 systolic murmur with an irregular tachycardic rhythm was auscultated on both sides of the chest. Systolic blood pressure was 140 mmHg. Panting was noticed in the hospital, but there was no crackle sound. Blood analysis revealed mild increases in liver panel levels (alanine aminotransferase 149 [reference interval, 19-70] U/L; and alkaline phosphatase, 185 [reference interval, 15-127] U/L) and severe increases in cardiac biomarker levels (n-terminal pro-brain natriuretic peptide, 4169 [reference interval, 50-900] pmol/L; and cardiac troponin I, 0.22 [reference interval, 0.03-0.12] ng/mL). On electrocardiography, irregularly irregular supraventricular tachycardic rhythm with an f-wave and no distinct p-wave was observed. Generalized cardiomegaly with an enlarged right atrium and left ventricle was confirmed on thoracic radiography. Moreover, hepatomegaly and an enlarged caudal vena cava were observed. Echocardiographic evaluation revealed a fibromuscular diaphragm in the right ventricle. Because of the obstructive lesion in the right ventricle, the right atrium and ventricle were enlarged (right atrial area index, 38.82 cm2/m2 [reference interval, 4.2-10.2 cm2/m2]; right ventricle end-diastolic area index, 14.152 cm2/m2 [reference interval, 4.9-10.92 cm2/m2]). Accordingly, the patient was diagnosed with double-chambered right ventricle (DCRV). Pimobendan, furosemide, enalapril, diltiazem, and S-adenosylmethionine (SAMe) were prescribed, and all symptoms were relieved. DCRV is a right-sided congenital heart defect resembling pulmonic valve stenosis. If symptoms are not severe, medical therapy can be facilitated without surgery or the balloon dilation.
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