Left ventricular rehabilitation in an infant with transposition of the great arteries, intact ventricular septum, pulmonary stenosis and small left ventricle.

We report the case of successful biventricular repair after left ventricular rehabilitation in an infant with transposition of the great arteries with an intact ventricular septum, pulmonary stenosis, a large atrial septal defect and a borderline small left ventricle (mitral annulus z-score: -3.6). This baby presented to us at 2 months of age after having a modified Blalock-Taussig shunt at another hospital. We restricted the atrial septal defect with the child on cardiopulmonary bypass. Ten weeks later, the mitral annulus z-score increased to -1.5, and the transpulmonary peak pressure gradient increased to 87 mmHg. Subsequently, we performed the aortic root translocation. The patient is currently an active 4-year-old boy.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications.

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Mechanical valves in the pulmonary position: An international retrospective analysis.

OBJECTIVE: Life expectancy of patients with congenital heart disease has improved over the past decades, increasing the need for a durable pulmonary prosthetic valve. Biological valves in various forms have become the valve of choice for pulmonary valve replacement (PVR), but structural valve deterioration is unavoidable in the long term. Use of a mechanical valve could be an alternative, but data on long-term outcomes are sparse. METHODS: We retrospectively collected and analyzed data on 364 patients with mechanical valves implanted in the pulmonary position between 1965 and 2014. The data originate from medical centers in Barcelona (Spain), Graz (Austria), Groningen (the Netherlands), Munich (Germany), Rochester (United States), Seoul (Republic of Korea), and Tehran (Iran). RESULTS: Median follow-up duration was 4.26 years (range, 0-27 years), mean age at implantation was 27.16 ± 12.2 years. Tetralogy of Fallot was the most common primary cardiac diagnosis, with a subgroup of 69.8%. Freedom from valvular thrombosis was 91% (95% confidence interval [CI], 87%-94%) at 5 years and 86% (95% CI, 81%-91%) at 10 years post-PVR. With a success rate up to 88%, thrombolysis was a successful therapy. Freedom from reoperation was 97% (95% CI, 94%-99%) at 5 years post-PVR and 91% (95% CI, 85%-95%) at 10 years. CONCLUSIONS: Mechanical PVR is associated with a limited risk of valvular thrombosis. Thrombolysis was an effective treatment in the majority.

Isolation of the Left Subclavian Artery with Right Aortic Arch in Association with Bilateral Ductus Arteriosus and Ventricular Septal Defect.

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.

Surgical modification for preventing a gothic arch after aortic arch repair without the use of foreign material.

OBJECTIVES: Systemic hypertension is the main late complication after arch reconstruction in patients with arch obstruction. Gothic arch geometry is suspected to be one of its possible causes. Accordingly, we evaluated here if a modified arch repair technique using an autologous pulmonary patch is effective in preventing gothic arch development. METHODS: Fifty infants who underwent arch repair with either a modified (n = 17) or conventional (n = 33) technique between January 2006 and August 2012 by a single surgeon were retrospectively reviewed. Arch geometry was compared using three categories (gothic, crenel or roman), classified by the height/width (H/W) ratio and the arch angle measured in computed tomography. RESULTS: No gothic arch geometry was observed in the modified group, whereas it was observed in 9 cases in the conventional group (P = 0.005). Moreover, reintervention for arch restenosis was performed only in the conventional group (n = 4; P = 0.29). No associated complications were observed, although the selective cerebral perfusion time was longer in the modified group than in the conventional group (28.5 ± 6.2 vs 17.1 ± 9.9 min; P < 0.001). Otherwise, there were no significant differences in clinical variables between the groups. The mean follow-up duration was 55.3 ± 26.7 months. Significant systemic hypertension was not observed in our study cohort. CONCLUSIONS: Our modified technique was proven to be not only highly effective in preventing gothic arch geometry, but also as equally safe in terms of early clinical outcomes as conventional arch reconstruction techniques.

Hybrid procedure for pulmonary atresia with ventricular septal defect in a low birth weight neonate.

Cardiac surgery in neonates with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight neonates with congenital heart disease continue to challenge the intellectual and technical skills of healthcare professionals. We present a case of a low-birth-weight neonate with pulmonary atresia and a ventricular septal defect, in whom palliation was achieved with a right ventricular outflow tract stent using a hybrid procedure.

Outcomes of mechanical valves in the pulmonic position in patients with congenital heart disease over a 20-year period.

BACKGROUND: Homografts or bioprosthetic valves have been preferred in the pulmonic position in patients with congenital heart disease. However, unsatisfactory long-term results have aroused interest in the use of mechanical valves. In this study, we investigated the long-term outcomes of mechanical valves implanted in the pulmonic position. METHODS: The medical records of 37 patients (27 male, 73%) who underwent 38 mechanical pulmonary valve replacements between October 1988 and February 2011 were reviewed, retrospectively. The median age of patients was 13.5 years (range, 7 months to 23 years), and the median number of prior operations per patient was 2 (range, 0 to 5). Tetralogy of Fallot was the most common diagnosis (n=23). The median valve size was 23 mm (range, 17 to 27 mm), and the median follow-up duration after pulmonary valve replacement was 24.6 months (range, 1.3 months to 22.5 years). Events were defined as the following: valve failure, thrombosis, embolism, bleeding, reoperation, and death. RESULTS: There was no in-hospital mortality, but there were 2 late deaths (1 heart failure and 1 traffic accident at 10.8 months and 8.7 years postoperatively, respectively). Excluding the traffic accident death, survival rates were 97%, 97%, and 97%, at 1, 5, and 10 years, respectively. Freedom from thromboembolism or bleeding events was 92%, 92%, and 78.8%, at 1, 5 and 10 years, respectively. Two reoperations were performed at 6.8 and 10.2 years postoperatively. Freedom from reoperation was 100%, 100%, and 85.7%, at 1, 5, and 10 years, respectively. CONCLUSIONS: Durability of mechanical valve in pulmonic position was excellent. Thromboembolism or bleeding events due to anticoagulation therapy were rare. In growing patients who have undergone prior sternotomies requiring a pulmonary valve replacement, a mechanical valve could be an attractive option.

Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy.

BACKGROUND: Protein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation. METHODS: Two hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5 years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records. RESULTS: Within the median follow-up duration of 5 years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2 years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2 years (range 0.21-7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p=0.0019), central venous pressure at postoperative 12 hours (p=0.0026), days of ICU stay (P=0.0449), days of hospitalization (p=0.0135), and days of chest tube indwelling (p=0.0493). Multivariate analysis, however, showed that only Cpv (p=0.0367) remained significant. The range of Cpv was 8.8-26.1 mm(2)/m(2)/mmHg (median 17.9) in patients with PLE, and 6.6-122.3 mm(2)/m(2)/mmHg (median 26.8) in patients without PLE. CONCLUSIONS: Low pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.

The importance of neo-aortic root geometry in the arterial switch operation with the trap-door technique in the subsequent development of aortic valve regurgitation.

OBJECTIVE: Regarding neo-aortic valve regurgitation (neo-AR) after the arterial switch operation (ASO), the 'trap-door' technique was supposed to be a risk factor due to a distortion of the sinotubular junction (STJ) geometry. Here we report our results of the 'trap-door' technique with a special emphasis on root geometry including the ratio of STJ to annulus. METHODS: From August 1991 to March 2010, 240 patients with transposition of the great arteries underwent the ASO and who had at least 1 year of follow-up were included in this study. The medical records were retrospectively reviewed. RESULTS: The median age and body weight at the time of operation were 11 (0-1213) days and 3.4 (1.30-18.75) kg, respectively. The median follow-up duration was 79 months (range 12 months-19.5 years). At the latest echocardiographic follow-up, only six patients had neo-AR greater than Grade II (6 of 240, 2.5%). We found no relationship between neo-AR greater than Grade II and perioperative factors. The actual sizes of the neo-aortic annulus, mid-sinus and STJ were observed as having increased over time. However, most z-scores of STJ at the latest echocardiography varied between -2 and 2 and, more importantly, the ratio of STJ to neo-aortic annulus was 0.93 ± 0.20, which was near normal at the latest echocardiographic follow-up. CONCLUSIONS: Our results showed a very low incidence of significant neo-AR, which was relatively attributable to the preserved z-score of STJ and the normal range of STJ/annulus ratio. Therefore, we propose that it is important to maintain these factors adequately during the reconstruction of the neo-aortic root in the ASO.

Mitral valve replacement using mechanical prostheses in children: early and long-term outcomes.

Compared with mitral repair, mitral valve replacement is an uncommon procedure in children due to associated high mortality and morbidity rates. The present study investigated early and late outcomes after MVR with mechanical prostheses in children at our institution. Between January 1994 and December 2009, 19 children underwent MVR. Mean patient age was 7.6 ± 5.5 years (range 3 months-16 years), and mean body weight was 23.7 ± 15.1 kg (range 5.0-58.1 kg). Mean prosthesis size was 25.8 ± 4.2 mm (range 19-31 mm). There were no operative or late mortalities. Three patients showed decreased left-ventricular function before surgery, and one of them underwent successful heart transplantation due to progressive LV dysfunction at 10 months after MVR. The proportion of patients with freedom from reoperation at 10 years was 94.7 ± 5%. There were no major thromboembolic or bleeding episodes. Although the small number of patients in our study was a limitation, MVR in children was found to result in excellent early and long-term outcomes. It appears that MVR could be considered in children before LV dysfunction develops.

Effectiveness and safety of percutaneous transcatheter implantation of pulmonary arterial stent in congenital heart disease.

BACKGROUND AND OBJECTIVES: Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. SUBJECTS AND METHODS: A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. RESULTS: Percent stenosis of stenotic lesions were decreased from 54.1±10.7% to 22.8±12.5% (p<0.001) and degree of decrement in affected lung perfusion was declined from 22.7±8.0% to 10.3±9.0% (p<0.001) immediately and lasts during mid-term follow up period. Complication rate relating the procedure was 12% (6 out of 12) and there was no mortality case. CONCLUSION: This series showed immediate and short term effectiveness of pulmonary arterial stent in congenital heart defects. We concluded that percutaneous transcatheter implantation of pulmonary arterial stent was safe and effective during short and mid-term follow up period.

Modified damus-kaye-stansel/dor procedure for a newborn with severe left ventricular aneurysm.

Congenital left ventricular aneurysm (CVA) is a rare cardiac malformation. The prognosis is variable, depending on such factors as the size in comparison to the ventricular cavity, signs of heart failure, arrhythmia and so on. Most infants and young children with large aneurysm showed poor clinical outcomes. Here, we report the case of patient who was prenatally diagnosed with a large CVA, who had severe left ventricular dysfunction at 21 weeks' gestation for which she successfully underwent a modified Damus-Kaye-Stansel/Dor procedure.

Left ventricular function after left ventriculotomy for surgical treatment of multiple muscular ventricular septal defects.

BACKGROUND: Optimal management of muscular ventricular septal defects (MVSD) is still not determined in the current era. Moreover, long-term left ventricular function after closure of MVSD is not well known. Thus, we investigated surgical outcomes including long-term left ventricular function after closure of MVSD through left ventriculotomy. METHODS: We conducted a retrospective review of medical records of 20 children who underwent MVSD closure between March 1993 and August 2010. There were 10 boys (50%) and 10 girls (50%). Patient age ranged from 1.6 to 103.4 months (median, 26.4 months), and body weight from 2.8 to 31.5 kg (median, 11.9 kg). Electrocardiogram results were normal sinus rhythm in all except 1 patient with congenital complete atrioventricular block. There were 16 patients who previously had palliative pulmonary artery banding procedures before closure of MVSD. There were 13 patients (65%) with Swiss-cheese type VSD. RESULTS: There was 1 hospital death of a patient with congenital complete atrioventricular block with pacemaker malfunction (5%). There was 1 late death of a patient with del 22q with adenoviral pneumonia. There was no reoperation. Median follow-up duration was 85.9 months (range, 4.7 to 166.7). The location of MVSD was apical portion in 10 patients (50%) and midtrabecular portion in 9 patients (45%). There were 6 Dacron patch closures and 13 direct closures of MVSD through left ventriculotomy. There was no complete atrioventricular block. Last follow-up echocardiographic data showed normal ejection fraction with 65.2% ± 8.2% after closure of MVSDs. There was no leakage in 8 patients; 11 patients had insignificant leakage, which disappeared spontaneously in 4 patients 17.9 months (median value) after operation. CONCLUSIONS: Our acceptable long-term results of left ventricular function after left ventriculotomy proved that this technique might be a viable option in the management of MVSD.

Heart transplantation in pediatric patients: twelve-year experience of the Asan Medical Center.

Heart transplantation is a standard treatment for end-stage heart disease. Pediatric heart transplantation, however, is not frequently performed due to the shortage of pediatric heart donors. This is the first report of pediatric heart transplantation in Korea. Our retrospective study included 37 patients younger than 18 yr of age who underwent heart transplantation at Asan Medical Center between August 1997 and April 2009. Preoperative diagnosis was either cardiomyopathy (n = 29, 78.3%) or congenital heart disease (n = 8, 22.7%). Mean follow up period was 56.9 ± 44.6 months. There were no early death, but 7 late deaths (7/37, 18.9%) due to rejection after 11, 15, 41 months (n = 3), infection after 5, 8, 10 months (n = 3), suspicious ventricular arrhythmia after 50 months (n = 1). There was no significant risk factor for survival. There were 25 rejections (25/37, 67.6%); less than grade II occurred in 17 patients (17/25, 68%) and more than grade II occurred in 8 patients (8/25, 32%). Actuarial 1, 5, and 10 yr survival was 88.6%, 76.8%, and 76.8%. Our midterm survival of pediatric heart transplantation showed excellent results. We hope this result could be an encouraging message to do more pediatric heart transplantation in Korean society.

Modified simple sliding aortoplasty for preserving the sinotubular junction without using foreign material for congenital supravalvar aortic stenosis.

OBJECTIVE: The surgical approach for treating supravalvar aortic stenosis (SVAS) has evolved from a plain patch technique to a three-dimensional patch repair, which has some drawbacks. Here, we report on the midterm outcomes after using our modified simple sliding aortoplasty preserving sinotubular junction without foreign material for surgical correction of SVAS. METHODS: Between June 2001 and February 2010, 18 children (median age, 6.2 years; range 0.5-2.2 years) with discrete SVAS underwent surgical repair. After a standard median sternotomy, cardiopulmonary bypass and cardioplegic arrest, the aorta was transected obliquely just distal to the point of stenosis. An incision was then made into the non-coronary sinus of the proximal aorta, and a counterincision was made into the lesser curvature of the ascending aorta, after which, the proximal and distal aorta were anastomosed directly with a running suture. Peak pressure gradients were estimated using echocardiography and the data were reviewed retrospectively. RESULTS: The median follow-up period was 39.6 months (range, 1-104.5 months). There was no early or late death. No patients required re-operation. Ten patients had Williams-Beuren syndrome. There were eight cases of concomitant pulmonary artery angioplasty. The mean pressure gradient decreased from 65.9 ± 18.4 mm Hg preoperatively to 15.2 ± 8.9 mm Hg at the final follow-up (P = 0.01). There was no significant, more than mild aortic regurgitation. CONCLUSIONS: Our modified simple sliding aortoplasty showed excellent surgical results, and may be a good option for discrete SVAS.

The outcome of open heart surgery for congenital heart disease in infants with low body weight less than 2500 g.

Although the outcome of neonatal cardiac surgery has dramatically improved, low body weight (LBW) is still considered an important risk for open heart surgery. The factors contributing to poor outcomes in LBW infants, however, are still unclear. We investigated risk factors for poor outcomes in infants weighing <2500 g who underwent surgical correction with cardiopulmonary bypass (CPB). From January 1995 to December 2009, 102 consecutive patients were included in this study. Median age and body weight at the time of surgery was 19 (range 1 to 365) days and 2.23 kg (range 1.3 to 2.5), respectively. Corrective surgery was performed on 75 infants. The median follow-up duration was 45.03 months (range 0.33 to 155.23). There were 23 (22.5%) hospital mortalities. Emergency surgery and low cardiac output (LCO) were associated with early mortality; however, body weight, Aristotle basic complex score, and type of surgery was not. Early morbidities, including delayed sterna closure, arrhythmia, and chylothorax, occurred in 39 (38.2%) infants. The overall actuarial survival rate at 10 years was 74.95% ± 4.37%. In conclusion, among infants weighing <2500 g who underwent open heart surgery with CPB, perioperative hemodynamic status, such as emergency surgery and LCO, strongly influenced early mortality. In contrast, LBW itself was not associated with patient morbidity or mortality.

Long term results of right ventricular outflow tract reconstruction with homografts.

BACKGROUND: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. MATERIALS AND METHODS: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was 79.2±14.8 months. RESULTS: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. CONCLUSION: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.

Mechanical circulatory support to control medically intractable arrhythmias in pediatric patients after cardiac surgery.

Postoperative intractable arrhythmia can result in high morbidity and mortality. This report describes our experiences using mechanical circulatory support (MCS) to control medically intractable arrhythmias in three pediatric patients with congenital heart disease (CHD), after palliative or total corrective open-heart surgery.

The effect and safety after extended use of continuous negative pressure of 75 mmHg over mesh and allodermis graft on open sternal wound from oversized heart transplant in a 3-month-old infant.

Negative pressure therapy (NPT) has been reported to be effective in treating infants with open chest wounds. This report further supports its effectiveness by treating a 3-month-old infant with a 12 × 7 cm sized opening in its chest after an oversized heart transplantation. After applying a mesh and allodermis over the defect, 75-mmHg continuous negative pressure was set and used for an extended period of 104 days. The haemodynamic status was evaluated during this period. The wound was closed with secondary intention and it healed well after NPT. There was no haemodynamic instability during the treatment course. The extended use of a continuous negative pressure of 75 mmHg over the mesh and alloderm graft was a reliable and safe option to close the massive defect in the chest of a 3-month-old infant.

Repair of atrial septal defect with Eisenmenger syndrome after long-term sildenafil therapy.

We report a woman with atrial septal defect and severe pulmonary hypertension with 25.0 Wood unit.m(2) of indexed total pulmonary vascular resistance. She underwent successful corrective repair of atrial septal defect after 2 years of treatment with sildenafil, and has been monitored for 4 years after repair. This case supports a "treat and repair" approach using advanced pulmonary vasodilator therapy in selected patients with inoperable severe pulmonary hypertension associated with atrial septal defect.