Surgical outcomes of pulmonary artery sling and congenital tracheal stenosis with right lung anomaly.

OBJECTIVES: This study aimed to investigate whether computed tomographic indices support surgical decision-making in patients with pulmonary artery sling, congenital tracheal stenosis and right lung underdevelopment. METHODS: A total of 38 patients with pulmonary artery sling and congenital tracheal stenosis underwent tracheoplasty. Patients were divided into 3 groups based on anatomical categorization: group normal lung (NL; n = 21), group H (right lung hypoplasia, n = 12) and group A (right lung agenesis or aplasia, n = 5). Using preoperative computed tomographic images, the severity of the tracheal bending due to the right posterior deviation of the aortic arch and the relative length of the left pulmonary artery for reimplantation was evaluated. RESULTS: Slide tracheoplasty posterior to the aortic arch with pulmonary artery reimplantation was performed in 32 patients (21, 10 and 1 in groups NL, H and A, respectively). Slide tracheoplasty anterior to the aortic arch was performed in 4 patients (2 patients each in groups H and A). Among the 6 patients with severe tracheal bending who underwent slide tracheoplasty posterior to the aortic arch, 5 required aortopexy for tracheomalacia. The overall mortality rate was 3% (group NL, n = 1). The relative length of the left pulmonary artery in group H (0.85) was significantly shorter than that in group NL (1.36, P < 0.0001). CONCLUSIONS: In patients with right lung underdevelopment, preoperative computed tomography elucidated the tracheal bending due to right posterior deviation of the aortic arch, which compromised tracheoplasty and shortness of the left pulmonary artery for pulmonary artery sling repair.

Selective Lesser Curvature Augmentation With Geometric Study for Repair of Aortic Arch Obstruction.

BACKGROUND: We repaired aortic coarctation and interrupted aortic arch with extended end-to-end anastomosis (EAA) through median sternotomy and performed lesser curvature augmentation with a pulmonary autograft patch (PAP) in selected patients with a long gap between anastomotic sites. We reviewed these outcomes and geometric implications. METHODS: All neonates and infants with biventricular morphology who underwent aortic arch reconstruction through median sternotomy between 2005 and 2019 were evaluated. Aortic arch geometry was analyzed with computed tomography routinely performed before and after surgery from 2009 on. RESULTS: There were 91 consecutive patients (median age, 1.2 months). Ten patients received PAP. One early death and no late deaths were noted. Overall survival was 98.9% at 10 years. Two left bronchomalacia and 1 recoarctation occurred in patients with EAA. Freedom from recoarctation was 97.4% at 10 years. We examined 68 patients with computed tomography. We used PAP in patients with a significantly longer gap between anastomotic sites indexed by the square root of the body surface area; its cutoff value was 29.0 mm/m (area under the curve, 0.86 mm/m). The PAP created a significantly greater arch angle (median, 91° versus 83°) and arch/descending diameter ratio (median, 1.2 versus 1.0) and preserved the arch width indexed by the square root of the body surface area (median, before surgery: 35.7 versus 34.4 mm/m; after surgery: 36.5 versus 29.9mm/m), compared with EAA. CONCLUSIONS: Aortic arch reconstruction with the current combined strategy provides satisfactory outcomes. Guided by geometric analysis, lesser curvature augmentation can be applied to patients who might experience recoarctation or airway compression with a directly anastomosed aortic arch.

Computed tomography-based surgical strategy for total anomalous pulmonary venous connection.

OBJECTIVES: We used computed tomographic angiography (CTA)-based surgical planning to clarify the anatomical indications of sutureless repair technique for total anomalous pulmonary venous connection. The mid-term impact of the current surgical strategies was evaluated. METHODS: One hundred twelve patients underwent repair for total anomalous pulmonary venous connection. The study period was divided into era 1 (1996-2010, n = 56) and era 2 (2011-2018, n = 56). Patients with single ventricular heart (SVH) were included. In era 2, the indications for primary sutureless repair and branch pulmonary vein incision were based on CTA findings. RESULTS: For patients with biventricular heart, the 5-year survival was 69% and 97% in eras 1 and 2, respectively (P = 0.0024). For patients with SVH, the 5-year survival was 21% and 70% in eras 1 and 2, respectively (P = 0.0007). During the follow-up period, the evidence of post-repair pulmonary vein stenosis (PVS) was observed in 12 patients with biventricular heart [era 1, 8 patients (23%); era 2, 4 patients (13%)], and 14 patients with SVH [era 1, 6 patients (60%); era 2, 8 patients (36%)]. Using multivariable analysis, preoperative CTA was associated with improved survival in both biventricular heart and SVH and associated with post-repair PVS-free survival in SVH. Since 2011, 12 patients with post-repair PVS underwent multiple reintervention with 1 recorded death (5-year survival: 88%). CONCLUSIONS: CTA-based surgical strategy for total anomalous pulmonary venous connection provided significant survival benefit. Although post-repair PVS could occur in era 2, aggressive reintervention appeared to be associated with improved survival and vein patency.

Left Atrial Decompression by Minithoracotomy During Extracorporeal Life Support.

Extracorporeal membrane oxygenation through cervical cannulation is an established option for pediatric patients with acute cardiopulmonary failure. However, left-sided heart decompression is sometimes mandatory in patients with severe left ventricular dysfunction. This report describes a fast and less invasive technique for placing a left atrial cannula through a left anterior minithoracotomy approach. In 4 critically ill children, this minimally invasive technique provided satisfactory left-sided heart decompression, and this report describes a representative case.

Pulmonary vascular disease in a failed Fontan patient with Down's syndrome.

It is well known that Down's syndrome is a strong risk factor for mortality after Fontan operations. We performed two lung biopsies in a Down's syndrome patient who underwent staged Fontan operations. The pathological findings revealed severe pulmonary arterial hypertrophy and a quantitative real-time polymerase chain reaction revealed the overexpression of endothelin and a decline in the eNOS level at the Fontan operation. Although the preoperative hemodynamic studies revealed that all of the criteria for Fontan had been fulfilled, the patient died of acute cardiac insufficiency, 35 days after the Fontan operation.

Endothelin-1 may play an important role in the Fontan circulation.

OBJECTIVES: Our goal was to evaluate whether endothlin-1 (ET-1) plays an important role in the Fontan circulation. METHODS: Thirteen patients with single-ventricle physiology (Glenn circulation, n = 7; Fontan circulation, n = 6) were evaluated using lung histopathological and immunohistochemical studies and then compared with the normal autopsied controls without congenital heart disease (n = 13). We evaluated the medial thickness of the small pulmonary arteries. For 10 of these patients, quantitative real-time polymerase chain reaction analyses of ET-1, endothelin receptors Type A and Type B, endothelin-converting enzyme-1 and endothelial nitric oxide synthase were performed. RESULTS: The medial thickness of the small pulmonary arteries in patients with single-ventricle physiology was greater than that of those in the control group (P = 0.0341). Severe medial hypertrophy of the pulmonary arteries was observed in patients who had poor outcomes. Immunohistochemical studies revealed that the marked expression of ET-1 was observed in the endothelium and media of their pulmonary arteries. In these patients, the messenger RNA expression of ET-1 was also increased. Two patients showed high levels of expression of ETAR and ETBR, although these 2 cases maintain good Fontan circulation. CONCLUSIONS: Medial hypertrophy and the overexpression of ET-1 in the pulmonary arteries were observed in some patients in whom the Fontan circulation failed. Our data suggest that ET-1 may play an important role in maintaining the Fontan circulation.

Anomalous origin of the right coronary artery evaluated with multidetector computed tomography and its clinical relevance.

BACKGROUND: Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly that may cause myocardial ischemia and sudden death. METHODS: We reviewed the clinicopathological records of three cases of AORCA, and compared these with two cases of sudden cardiac death with AORCA revealed by autopsy. RESULTS: We report three juvenile cases with an AORCA originating above the commissural junction between the left and right aortic sinuses, with interarterial and intramural compression. They presented with exertional symptoms and were diagnosed with an AORCA by multidetector computed tomography (MDCT), which successfully delineated the spatial resolution of the anomalous origin and course of the right coronary artery (RCA), in the operating room. All three underwent successful surgical unroofing of the RCA. Two cases of sudden cardiac death with AORCA revealed by autopsy showed a slit-like orifice, acute-angled take-off, and long intramural course of the RCA, resembling the RCAs of three juvenile cases. CONCLUSIONS: It is crucial to be alert to the presentation of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery, such as those observed in these three cases. MDCT provided an excellent definition and spatial resolution of the unusual origin and intramural course of the RCA, facilitating the correct surgical remedy and resulting in a good outcome for the patients.

Current topics in surgery for multiple ventricular septal defects.

In this review article, we describe several topics, including the sandwich technique, the transatrial re-endocardialization technique, the limited apical left ventriculotomy approach and device closure. The sandwich technique was introduced for the closure of muscular ventricular septal defects (VSD) by sandwiching the septum between two felt patches placed in the left and right ventricle. This technique requires neither the transection of muscular trabeculae nor ventriculotomy. Although the sandwich technique has resulted in the improvement of surgical outcomes, cases of postoperative cardiac dysfunction have been reported. Multiple smaller VSDs have been closed with transatrial re-endocardialization. Septal dysfunction may be avoided through this technique, in which the septal trabeculae are approximated in two layers of superficial, endocardial running sutures. Recently, a number of reports have recommended a limited apical left ventriculotomy approach. With this technique, a much shorter incision of around 1 cm at the apex of the left ventricle may be sufficient for achieving the complete closure of apical muscular VSDs. The transcatheter or perventricular device closure of muscular VSDs has increasingly been performed with good results. Although favorable early and mid-term results of device closure have been reported, this method is not always safer or less invasive than surgical closure. Long-term evaluations should be performed to determine whether the right and left ventricular functions are affected by treatment with relatively large devices in the heart.

Follow-Up of Persistent Tracheal Stenosis After Surgery for a Double Aortic Arch.

Persistent respiratory symptoms often occur after surgical repair of double aortic arch (DAA). Most often, symptoms are relatively mild and tend to be self-limited and improve with growth. Multidetector computed tomography (MDCT) imaging can be used to obtain needed anatomic information regarding the potential for extrinsic airway compression and is minimally invasive, safe, and readily available after surgery. We herein report the cases of two patients with persistent airway symptoms after surgical therapy for a double aortic arch. One of the patients eventually required aortopexy in order to achieve complete relief from recurrent symptoms of airway compression. In the other case, wheezing persisted following surgical division of the vascular ring, but it resolved over time without further intervention. In both cases, serial follow-up imaging with computed tomography scans was informative and helped guide management decisions.

Current topics in surgery for isolated total anomalous pulmonary venous connection.

Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains a challenge, with reported early mortality rates of up to 20%. In this review article, we describe several topics, including surgery for neonates, diagnoses with multidetector computed tomography (MDCT), and primary sutureless repair. Several studies have reported mortality rates of around 10%, and demonstrated unchanged hospital mortality in neonates, despite improvement of the overall mortality of cohorts including older patients. Previous reports identified a low body weight at the time of the operation, preoperative pulmonary venous obstruction (PVO), and a prolonged cardiopulmonary bypass time as risk factors for hospital mortality. With the development of new technologies, MDCT has become a good diagnostic modality for use in the pre- and post-operative evaluation. MDCT delineates the drainage site of the vertical vein and the atypical vessel into the systemic vein, and it can also evaluate the existence of obstruction in the vertical vein. Following favorable experiences with post-repair PVO, the indications for sutureless repair as a primary operation have been expanded for infants, including those at risk of developing PVO after the repair of TAPVC. Primary sutureless repair has proven especially useful for difficult patient groups, such as those with congenital PVO, infracardiac TAPVC with small pulmonary veins, or mixed-type TAPVC.

N-terminal pro-brain natriuretic peptide as a predictor of reoperation in children with surgically corrected tetralogy of fallot.

BACKGROUND: Most patients with surgically corrected tetralogy of Fallot (TOF) are faced with multiple residua and sequelae such as pulmonary regurgitation (PR), resulting in reoperation for pulmonary valve replacement (PVR). Plasma brain natriuretic peptide (BNP) level and serum N-terminal pro-BNP (NT-pro-BNP) level are useful as diagnostic objective markers of chronic heart failure (CHF). The aim of the study was to examine whether these markers have predictive ability for reoperation in children with surgically corrected TOF. METHODS AND RESULTS: Fifty-eight patients (38 male, 20 female) aged 1-18 years (median, 7 years) were enrolled. Serum NT-pro-BNP in TOF patients was significantly higher than in age-matched hospital controls without CHF (359.5±449.7pg/ml vs. 86.1±45.1pg/ml, respectively; P<0.0001). BNP and NT-pro-BNP had a better correlation with CHF index, RVEDP, and LVEDV in TOF groups. Children with surgically corrected TOF who had indication for PVR had higher BNP and NT-pro-BNP and more severe PR than those without indication for PVR. On multivariate logistic regression analysis, NT-pro-BNP was the strongest predictor for reoperation in patients with surgically corrected TOF. Area under the curve of NT-pro-BNP for reoperation was 0.950 (P<0.001) with a sensitivity of 88.9% and specificity of 91.8%. CONCLUSIONS: NT-pro-BNP is a good biomarker for monitoring CHF, and is a good predictor of PVR in children with surgically repaired TOF.

Ventricular septal dysfunction after surgical closure of multiple ventricular septal defects.

BACKGROUND: We assessed the global and regional ventricular septal functions using conventional echocardiography and two-dimensional speckle tracking imaging in children with postoperative multiple ventricular septal defects. METHODS: Thirty-six children were studied: 16 with postoperative multiple ventricular septal defects and 20 normal control subjects. In children with multiple ventricular septal defects, 60 ventricular septal defects were closed using one of three different techniques (patch closure, the sandwich technique, direct closure). Speckle tracking imaging was applied to three short-axis echocardiographic images. RESULTS: The total patch area used in the multiple ventricular septal defects group was correlated with the postoperative ejection fraction (r=0.703) and Tei index (r=0.778). The global septal peak systolic radial displacement and global septal peak systolic radial strain in the multiple ventricular septal defects group were significantly lower than those observed in the control subjects. The peak systolic radial strain in the segments closed with patches and the peak systolic radial displacement in the segments closed with the felt sandwich technique were significantly lower than those observed in the intact septal segments. No significant regional functional depressions were identified in the segments that were closed directly. CONCLUSIONS: The postoperative ventricular global and septal functions were significantly reduced in children with multiple ventricular septal defects, especially in the cases with complex congenital heart disease and that were closed with large prosthetic materials. These results suggest that an effort to minimize the use of patch materials may lead to preserved postoperative ventricular function.

Congenital sternal cleft.

We describe a rare case of congenital sternal cleft. Multidetector computed tomography showed the counterclockwise rotation of the heart. Primary surgical correction was successfully performed using the Sabiston technique when the patient was 20 days old. Although the circulatory and respiratory status was unstable in the immediate postoperative period, this abated, and the patient was discharged without complications on the 13th postoperative day. Postoperative multidetector computed tomography showed that the heart approached the normal position.

Management of pulmonary venous obstruction.

Although improved surgical techniques have led to significantly better outcomes of surgery for total anomalous pulmonary venous connection, the risk of progressive pulmonary venous obstruction continues to be a clinical problem. Both obstructed total anomalous pulmonary venous connection and post-repair pulmonary venous obstruction are associated with a significant risk of recurrent obstruction or death, requiring reoperation for stenosis. In general, side to side anastomosis of the pulmonary venous confluence to the functional left atrium has been performed for supracardiac and infracardiac total anomalous pulmonary venous connection. Repair of total anomalous pulmonary venous connection to the coronary sinus invariably involved unroofing the coronary sinus, followed by pericardial patch closure of the atrial septal defect. Recently, sutureless technique has been adopted as the primary operation for the subgroups of patients that are thought to be at high risk for post-repair pulmonary venous obstruction, such as those with total anomalous pulmonary venous connection associated with right isomerism, infracardiac total anomalous pulmonary venous connection with small individual pulmonary veins, or mixed-type total anomalous pulmonary venous connection. Because the sutureless technique does not require direct anastomosis to the confluence, aggressive resection of the obstructed pulmonary venous tissue can be achieved, and surgically induced distortion of the suture line can be avoided, which may help to prevent subsequent pulmonary venous obstruction. Conventional management strategies for recurrent pulmonary venous obstruction have typically been associated with poor outcomes. Recent reports have supported the use of the sutureless technique to treat post-repair pulmonary venous obstruction.

Pseudoaneurysm following percutaneous balloon angioplasty for aortic arch recoarctation after the Norwood procedure.

We experienced a rare case of 5-month-old male infant presenting with a pseudoaneurysm following percutaneous balloon angioplasty for aortic arch recoarctation after undergoing the Norwood procedure. The pseudoaneurysm, which measured 1 cm in diameter, was located between the left carotid artery and the left subclavian artery. Under deep hypothermic circulatory arrest, resection of the pseudoaneurysm and reconstruction of the neoaortic arch were performed successfully.

Modified primary sutureless repair of total anomalous pulmonary venous connection in heterotaxy.

Early and late mortality remain high after palliative open heart surgery in low-weight neonates. In addition, the need for neonatal repair and the presence of pulmonary venous obstruction are risk factors for mortality in right isomerism. We describe a modified primary sutureless repair of infracardiac total anomalous pulmonary venous connection in a 1,600 g neonate with heterotaxy. Endarterectomy of intimal hyperplasia localized to the anastomotic site was required at bilateral bidirectional cavopulmonary connection 6 months after the initial repair. The patient underwent a successful fenestrated Fontan procedure using an extracardiac conduit at the age of 2 years.

Successful replacement of aortic root with valve-sparing technique and proximal arch in a patient with myelodysplastic syndrome.

Cardiac surgery in a patient with myelodysplastic syndrome (MDS) increases the risk of bleeding and infection. Here we report a case of a 70-year-old man with MDS who underwent successful replacement of the aortic root with the valve-sparing technique and proximal arch for aneurysmal dilatation from the aortic root to ascending aorta with moderate aortic valve regurgitation. Perioperatively, a transfusion of red blood cells and an infusion of a grannulocyte colony-stimulating factor were required for his serious erythrocytopenia and leukocytopenia. Bleeding tendency was so severe that re-exploration to control postoperative surgical bleeding was performed and a large amount of blood cells were transfused. There was no infection on the postoperative course. Perioperative management for cardiac surgery in patients with MDS must be carefully programmed by a co-operative team consisting of cardiovascular surgeons and hematologists.