• Journal of Chest Surgery
• /
• v.56 no.3
• /
• pp.155-161
• /
• 2023

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m², p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

• Journal of Chest Surgery
• /
• v.9 no.2
• /
• pp.220-238
• /
• 1976

Six cases of congenital heart disease were operated on by means of cardiopulmonary bypass between December, 1975 and April, 1976. Two cases of ventricular septal defects (VSD), two cases of VSD, associated with ruptured aneurysm of sinus Valsalva, two cases of atrial septal defects (ASD) and one case of pulmonic stenosis with patent ductus arteriosus were operated. Sarns roller pumps and Bentley Temptrol oxygenators were used for extracorporeal circulation. Pump oxygenator was primed with Ringer's lactate solution, 5% dextrose in water, mannitol, and ACD blood. Flow rate ranged from 2.0 to $2.4L/M^2/min$. Bicarbonate was added to the oxygenator with estimated amount as 15 mEq/L/hr. Venous catheters were introduced into superior and inferior vena cava, and oxygenated blood was returned to the body through aortic cannula inserted into ascending aorta. Moderate hypothermia ($30^{\circ}C$) was induced by core cooling. Aorta was cross clamped for 15 minutes and released for 3 minutes, and repeated clamping when necessary. Atrial and ventricular septal efects were closed by direct sutures. Aneurysms of sinus Valsalva ruptured into the right ventricle were repaired through right ventriculotomy by d:rect closure with Dacron patch reinforcement. Cardiopulmonary bypass time varied from 66 to 209 minutes, and aorta cross clamping time ranged from 13 to 56 minutes. Postoperative bleeding was minimal except one case who needed for evacuation of substernal hematoma. Intra- and postoperative urinary output was satisfactory. Acid-base balance, partial pressure of $O_2$, electrolytes, and hematological changes during intra- and post-perfusion period remained at the acceptable ranges. No mortality was experienced.

• Journal of Genetic Medicine
• /
• v.16 no.2
• /
• pp.71-75
• /
• 2019

Periventricular nodular heterotopia (PNH) is a malformation of cortical development in which normal neurons inappropriately cluster in periventricular areas. Patients with Mowat-Wilson syndrome (MWS) typically present with facial gestalt, complex neurologic problems (e.g., severe developmental delay with marked speech impairment and epilepsy), and multiple anomalies (e.g., Hirschsprung disease, urogenital anomalies, congenital heart defects, eye anomalies, and agenesis of the corpus callosum [CC]). MWS is mostly caused by haploinsufficiency of the gene encoding zinc-finger E-box-binding homeobox 2 (ZEB2) due to premature stops or large deletions. We present a case report of a 9-year-old girl with PNH, drug-responsive epilepsy, severe intellectual disability, and facial dysmorphisms only in whom we performed whole-exome sequencing and found a de novo heterozygous missense mutation (c.3134A>C; p.His1045Pro) of ZEB2 (NM_014795.3; NP_055610.1). This mild case of MWS caused by a rare novel missense mutation of ZEB2 represents the first report of MWS with isolated PNH.

• Journal of Chest Surgery
• /
• v.28 no.3
• /
• pp.237-246
• /
• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8 kg [mean weight, 4.0 1.4 kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1 $\pm$ 3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients [86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septum was resected whenever necessary. There were seven early deaths [16.8 % , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis, stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily. We concluded that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are reasonable.

• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.127-131
• /
• 2010

Supernumerary derivative (22) syndrome is a rare genomic syndrome. It is characterized by severe mental retardation, microcephaly, failure to thrive, preauricular tag or sinus, ear abnormalities, cleft and/or high-arched palate, micrognathia, kidney abnormalities, congenital heart defects, and genital abnormalities in males. In 99% of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. To date, there have been about 100 case reports of supernumerary derivative (22) syndrome. In most of the cases, supernumerary derivative (22) syndrome was the result of 3:1 meiotic segregation in the maternal 11;22 translocation carrier. We now report a case of 47,XX, + der(22)t(11;22)(q23;q11.2) resulting from 3:1 meiotic segregation of the paternal translocation carrier.

• Journal of Chest Surgery
• /
• v.27 no.5
• /
• pp.353-363
• /
• 1994

From January 1978 to December 1992, 59 patients of double chambered right ventricle were repaired. Surgical correction consisted of closure of the ventricular septal defect and resection of anomalous muscle bundles through right ventriculotomy [Group I ; 34 patients] or right atriotomy [Group II ; 25 patients]. Between these two groups, there was no difference in the operation time and the postoperative results. All patients survived. In group I, hemodynamically significant residual ventricular septal defect was found in three and reoperations were necessary. In one patient, subacute bacterial endocarditis developed postoperatively. In group II, complete atrioventricular block developed in one and mediastinitis in two. Follow-up period was from 2 to 75 months [mean 17.1 months]. There was no late death. All patients have remained in sinus rhythm except one patient. Careful evaluation of echocardiographic and catheterization data preoperatively and careful examination of the anatomy intraoperatively are necessary so that double chambered right ventricle should not be overlooked, because most ventricular septal defects are now closed through the right atrium. Repair of double chambered right ventricle is also easily performed through the atrial approach. Transatrial repair should be considered as an alternative to the transventricular approach in patients with this congenital heart defect. Successful surgical correction of double chambered right ventricle is expected with excellent long term results.

• Journal of Veterinary Clinics
• /
• v.27 no.6
• /
• pp.735-739
• /
• 2010

An 8 month old female Maltese (body weight 3.6 kg) was referred with primary complaints of dyspnea and exercise intolerance. Diagnostic imaging studies revealed marked cardiomegaly and prominent main pulmonary trunk dilation on thoracic radiography, abnormally arisen aortic roots (toward right ventricle) with left-to right shunted perimembraneous ventricular septal defect located underneath of aortic root, aortic root was located to predominantly to the right ventricle and pulmonary regurgitation (peak velocity 4.7 m/s, pressure gradient ~88 mmHg) from pulmonary over-circulation and hypertension on echocardiography, indicating double outlet right ventricle (DORV). The dog was treated with furosemide (1 mg/kg, BID) for reducing volume overload at right ventricle, spironolatcone (1 mg/kg) and enalapril (0.5 mg/kg) for minimizing deleterious cardiac remodeling, and sildenafil (1 mg/kg) for lessening pulmonary over-circulation and hypertension. The clinical condition of this dog was improved after 1 week of medical treatment. The dog is currently survived and regularly monitored.

• Journal of Chest Surgery
• /
• v.46 no.2
• /
• pp.93-97
• /
• 2013

Background: Minimally invasive cardiac surgery has emerged as an alternative to conventional open surgery. This report reviews our experience with atrial septal defect using the da VinciTM surgical robot system. Materials and Methods: This retrospective study included 50 consecutive patients who underwent atrial septal defect repair using the da VinciTM surgical robot system between October 2007 and May 2011. Among these, 13 patients (26%) were approached through a totally endoscopic approach and the others by mini-thoracotomy. Nineteen patients had concomitant procedures including tricuspid annuloplasty (n=10), mitral valvuloplasty (n=9), and maze procedure (n=4). The mean follow-up duration was $16.9{\pm}10.4$ months. Results: No remnant interatrial shunt was detected by intraoperative or postoperative echocardiography. The atrial septal defects were mainly repaired by Gore-Tex patch closure (80%). There was no operative mortality or serious surgical complications. The aortic cross clamping time and cardiopulmonary bypass time were $74.1{\pm}32.2$ and $157.6{\pm}49.7$ minutes, respectively. The postoperative hospital stay was $5.5{\pm}3.3$ days. Conclusion: The atrial septal defect repair with concomitant procedures like mitral valve repair or tricuspid valve repair using the da VinciTM system is a feasible method. In addition, in selected patients, complete port access can be helpful for better cosmetic results and less musculoskeletal injury.

• Journal of Chest Surgery
• /
• v.46 no.2
• /
• pp.98-103
• /
• 2013

Background: Atrial fibrillation (AF) is a common complication in elderly patients with atrial septal defect (ASD). The purpose of this study was to examine the efficacy of the maze procedure in these patients. Materials and Methods: Between February 2000 and May 2011, 46 patients underwent the maze procedure as a concomitant operation with ASD closure. Three patients who underwent a right-sided maze were excluded, and one patient was lost to follow-up. The mean follow-up duration was $3.2{\pm}2.5$ years. Electrocardiography was performed 1 month, 3 months, 6 months, and 1 year after surgery, and checked annually after that. Results: AF persisted in 4 patients after surgery. One year after surgery, among 38 patients, 55.3% remained in sinus rhythm without antiarrhythmic drugs. However, when including the patients who took antiarrhythmic drugs, 92.1% were in sinus rhythm. Freedom from AF recurrence at 3 months, 6 months, 1 year, 2 years, 3 years, and 5 years after surgery were $97.4{\pm}2.6$, $94.4{\pm}3.8$, $91.2{\pm}4.9$, $87.8{\pm}5.8$, $79.5{\pm}7.6$, and $68.2{\pm}12.4$, respectively. There was no early mortality after operation. Conclusion: Concomitant treatment with the maze procedure and ASD closure is safe and effective for restoring the sinus rhythm.

• Journal of Chest Surgery
• /
• v.41 no.6
• /
• pp.747-750
• /
• 2008

Although surgical closure is the standard approach for a muscular ventricular septal defect, the procedure may be complicated by poor visualization and the need for incision on the ventricle. Another approach is, catheter-based intervention. However, it also has limitations. A hybrid procedure, the intraoperative combined use of an interventional device may reduce the procedure's invasiveness. We successfully managed two cases of muscular ventricular septal defect with a hybrid procedure. We report here on these 2 cases along with a review of the literature.