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.

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.

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.

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.

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.

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.

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.

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.

The significance of pulmonary annulus size in the surgical management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis.

OBJECTIVE: Aortic translocation has received growing attention in the management of complete transposition with ventricular septal defect and pulmonary stenosis, but the criteria regarding pulmonary stenosis for selecting this option have yet to be established. The aim of this study is to evaluate the significance of pulmonary annulus size with the outcome after the arterial switch operation. METHODS: Between November 1996 and September 2008, 250 patients underwent the arterial switch operation for complete transposition. Among them, 8 patients with a pressure gradient greater than 30 mm Hg, bicuspid pulmonary valve, and an aortic Z-score of the pulmonary annulus less than 0 were included in this retrospective study. The median age was 19.1 months (range, 0.5-80.0 months). The median follow-up was 39.7 months (range 9.1-139.5 months). RESULTS: At latest follow-up, the Z-score of the neoaortic annulus increased from -1.50 + or - 1.13 (range, -3.42 to -0.35) to 1.10 + or - 1.15 (range, -0.8 to 2.10) (P < .01). No patient had a significant pressure gradient across the left ventricular outflow tract. There was 1 early death and there were no late deaths. Two reoperations were performed in 1 patient for neoaortic stenosis at 81 months and 110 months after the operation. Latest echocardiogram revealed grade 0 or 1 neoaortic insufficiency. CONCLUSION: It was possible to extend the indication for the arterial switch operation with acceptable outcome to the patient with a Z-score of about -3 of the pulmonary annulus despite bicuspid pulmonary valve. Inasmuch as the arterial switch operation has benefits over the other options, a large-scale study is required for more reasonable triage in this group of patients.

CT findings of plastic bronchitis in children after a Fontan operation.

BACKGROUND: Plastic bronchitis is a rare cause of acute obstructive respiratory failure in children. Life-threatening events are much more frequent in patients with repaired cyanotic congenital heart disease, and most frequent following a Fontan operation. Commonly, the diagnosis is not made until bronchial casts are expectorated. Detailed CT findings in plastic bronchitis have not been described. OBJECTIVE: To describe the CT findings in plastic bronchitis in children after a Fontan operation. MATERIALS AND METHODS: Three children with plastic bronchitis after a Fontan operation were evaluated by chest CT. Bronchial casts were spontaneously expectorated and/or extracted by bronchoscopy. Airway and lung abnormalities seen on CT were analyzed in the three children. RESULTS: CT demonstrated bronchial casts in the central airways with associated atelectasis and consolidation in all children. The affected airways were completely or partially obstructed by the bronchial casts without associated bronchiectasis. The airway and lung abnormalities rapidly improved after removal of the bronchial casts. CONCLUSION: CT can identify airway and lung abnormalities in children with plastic bronchitis after a Fontan operation. In addition, CT can be used to guide bronchoscopy and to monitor treatment responses, and thereby may improve clinical outcomes.

Ruptured sinus of Valsalva aneurysm: transaortic repair may cause sinus of Valsalva distortion and aortic regurgitation.

OBJECTIVES: Recurrent or newly developing aortic regurgitation is a critical problem after the repair of ruptured sinus of Valsalva aneurysm. METHODS: A retrospective review of 56 patients who underwent surgical repair of ruptured sinus of Valsalva aneurysm between June 1990 and August 2006 was performed. Rupture of the right coronary sinus into the right ventricle was the most common anatomic type (39/56, 69.6%). Preoperative aortic regurgitation equal to or greater than grade II (n = 8, 17.9%) was managed by repair (aortic valvuloplasty, n = 5) or replacement (n = 3). Ruptured sinus of Valsalva aneurysm was repaired primarily (n = 7) or by patching (n = 10) through an aortotomy in 17 patients (transaortic group). In the remaining patients (n = 39), ruptured sinus of Valsalva aneurysm was repaired primarily from the chamber into which the corresponding aortic sinus ruptured, and the aneurysmal sac was reinforced with a supporting patch (non-transaortic group). RESULTS: Median follow-up duration was 46 months (0.4-177 months). There were 2 late deaths. Excluding 3 patients with aortic valve replacement on aneurysm repair, 11 patients (11/53, 21%) had recurrent or new-onset significant aortic regurgitation (> or = II/IV) during the follow-up period. By multivariable analysis, aortic valvuloplasty at initial operation was the only significant risk factor for postoperative aortic regurgitation (P < .001). After adjustment, the non-transaortic approach appeared to be associated with a lower risk of postoperative aortic regurgitation, with marginal significance (hazard ratio 0.28; P = .058). Five-year freedom from significant aortic regurgitation in the transaortic and non-transaortic groups was 68% +/- 12% and 94% +/- 4%, respectively. CONCLUSION: Transaortic repair of ruptured sinus of Valsalva aneurysm may cause postoperative aortic regurgitation by progressive distortion of the aortic sinus geometry.

Perioperative evaluation of airways in patients with arch obstruction and intracardiac defects.

BACKGROUND: Patients with arch obstruction and intracardiac defects have a high probability of abnormal aortopulmonary space geometry, which provides airway compression. The tissue-to-tissue technique arch repair could result in real airway problems. This report describes our experience with the perioperative evaluation and management of airway problems. METHODS: We retrospectively reviewed the medical records of 90 patients with arch obstruction and intracardiac defects who underwent computed tomography (CT) and corrective surgery in our institution between January 2000 and January 2007. RESULTS: Of the 77 patients who underwent preoperative CT (group 1), 21 were found to have airway compression (27.2%). Of those 21 patients, 5 underwent concomitant airway relieving procedures. In group 1, 2 patients required subsequent secondary surgery for airway problems after the initial arch repair. Of the 13 patients who underwent postoperative CT only (group 2), 6 underwent subsequent secondary surgery for airway relief. For airway relief, several procedures were additionally performed (eg, right pulmonary artery translocation anterior to the aorta, aortopexy, peribronchial dissection, and tissue augmentation). In terms of the type of arch repair, 48 patients underwent end-to-side anastomosis, 39 underwent extended end-to-end anastomosis, and 3 underwent end-to-end anastomosis. End-to-side was the repair type most commonly associated with airway compression requiring additional procedure (10 of 15, 66.6%). CONCLUSIONS: Patients with arch obstruction and intracardiac defects had a rather high incidence of airway compression preoperatively and postoperatively. Preoperative CT and intraoperative complementary bronchoscopy were useful for identifying and fixing the airway problems. Additional procedures for relieving airway compression were required more frequently after end-to-side type arch repair than after extended end-to-end anastomosis. More meticulous intraoperative evaluation and management are recommended in this type of repair.

Multislice CT angiography of interrupted aortic arch.

Interrupted aortic arch (IAA) is defined as complete luminal and anatomic discontinuity between the ascending and descending aorta. Because almost all patients with IAA become critically ill during the neonatal period, they should undergo urgent corrective surgery. This clinical urgency necessitates a fast and accurate noninvasive diagnostic method. Although echocardiography remains the primary imaging tool for this purpose, it is not always sufficient for planning surgical correction of IAA, principally due to a limited acoustic window and the inexperience of imagers. In this context, multislice CT angiography is regarded as an appropriate imaging technique complementary to echocardiography because it is fast, accurate, and objective for the diagnosis of IAA. In this article we describe what cardiac radiologists should know about IAA in their clinical practice, including clinicopathological features, CT features with contemporary surgical methods and postoperative complications, and differentiation from coarctation of the aorta and aortic arch atresia.

Reverse Blalock-Taussig shunt facilitates the growth of the ascending aorta after hybrid palliation.

A 13-day-old baby girl with tricuspid atresia (IIc), who was prematurely born at 32 weeks and 5 days of gestation and weighed 2.2 kg, underwent bilateral pulmonary artery banding, ductal stenting, and reverse Blalock-Taussig shunt. Cardiac computerized tomography at 4 months postoperatively showed that the ascending aorta outgrew the somatic growth, presumably thanks to the forward flow through the reverse Blalock-Taussig shunt. At 6 months postoperatively, the patient underwent a successful second-stage operation.