Isolated Ventricular Inversion: A Rare Complex Congenital Heart Disease With Neonatal Cyanosis.

A 1-day-old girl was referred for a cardiology consultation for a mean saturation at 80% without respiratory distress. Echocardiography showed an isolated ventricular inversion. This entity is extremely rare, with fewer than 20 cases reported. This case report describes the clinical evolution and the complex surgical management of this pathology. (Level of Difficulty: Advanced.).

An Unusual Case of Left Ventricular Outflow Tract Pseudoaneurysm.

Left ventricular outflow tract (LVOT) pseudoaneurysm is a rare condition with a wide range of causes and various clinical outcomes. The causes range from infections, trauma to the chest wall, and iatrogenic origins. We present a unique case of idiopathic LVOT pseudoaneurysm in a patient with no obvious clinical risk factors. (Level of Difficulty: Advanced.).

Three-dimensional printing and virtual reconstruction in surgical planning of double-outlet right ventricle repair.

Objectives: For more than a decade, 3-dimensional (3D) printing has been identified as an innovative tool for the surgical planning of double-outlet right ventricle (DORV). Nevertheless, lack of evidence concerning its benefits encourages us to identify valuable criteria for future prospective trials. Methods: We conducted a retrospective study involving 10 patients with DORV operated between 2015 and 2019 in our center. During a preoperative multidisciplinary heart team meeting, we harvested surgical decisions following a 3-increment step process: (1) multimodal imaging; (2) 3D virtual valvular reconstruction (3DVVR); and (3) 3D-printed heart model (3DPHM). The primary outcome was the proportion of predicted surgical strategy following each of the 3 steps, compared with the institutional retrospective surgical strategy. The secondary outcome was the change of surgical strategy through 3D modalities compared with multimodal imaging. The incremental benefit of the 3DVVR and 3DPHM over multimodal imaging was then assessed. Results: The operative strategy was predicted in 5 cases after multimodal imaging, in 9 cases after 3DVVR, and the 10 cases after 3DPHM. Compared with multimodal imaging, 3DVVR modified the strategy for 4 cases. One case was correctly predicted only after 3DPHM inspection. Conclusions: 3DVVR and 3DPHM improved multimodal imaging in the surgical planning of patients with DORV. 3DVVR allowed a better appreciation of the relationships between great vessels, valves, and ventricular septal defects. 3DPHM offers a realistic preoperative view at patient scale and enhances the evaluation of outflow tract obstruction. Our retrospective study demonstrates benefits of preoperative 3D modalities and supports future prospective trials to assess their impact on postoperative outcomes.

Cardiac surgery in children with trisomy 13 or trisomy 18: How safe is it?

Objective: Surgery for heart defects in children with trisomy 13 or 18 is controversial. We analyzed our 20-year experience. Methods: Since 2002, we performed 21 operations in 19 children with trisomy 13 (n = 8) or trisomy 18 (n = 11). Age at operation was 4 days to 12 years (median, 154 days). Principal diagnosis was ventricular septal defect in 10 patients, tetralogy of Fallot in 7 patients, arch hypoplasia in 1 patient, and patent ductus arteriosus in 1 patient. Results: The initial operation was ventricular septal defect closure in 9 patients, tetralogy of Fallot repair in 7 patients, pulmonary artery banding in 1 patient, patent ductus arteriosus ligation in 1 patient, and aortic arch/coarctation repair in 1 patient. There were no operative or hospital deaths. Median postoperative intensive care and hospital stays were 189 hours (interquartile range, 70-548) and 14 days (interquartile range, 8.0-37.0), respectively, compared with median hospital stays in our center for ventricular septal defect repair of 4.0 days and tetralogy of Fallot repair of 5.0 days. On median follow-up of 17.4 months (interquartile range, 6.0-68), 1 patient was lost to follow-up after 5 months. Two patients had reoperation without mortality. There have been 5 late deaths (4 with trisomy 18, 1 with trisomy 13) predominately due to respiratory failure from 4 months to 9.4 years postoperatively. Five-year survival was 66.6% compared with 24% in a group of unoperated patients with trisomy 13 or 18. Conclusions: Cardiac operation with an emphasis on complete repair can be performed safely in carefully selected children with trisomy 13 or trisomy 18. Hospital resource use measured by postoperative intensive care and hospital stays is considerably greater compared with nontrisomy 13 and 18.

From Double-Chambered Right Ventricle to Double-Chambered Left Ventricle: Unusual Evolution of a Ventricular Septal Defect.

We present the first imaging registry of the progressive isolation of an apical chamber of the right ventricle caused by the hypertrophy of the moderator band generated from the hemodynamic effect of a ventricular septal defect, leaving the apex of the right ventricle as an accessory chamber of the left ventricle. (Level of Difficulty: Advanced.).

Device Occlusion of Native Pulmonary Blood Flow After Cavopulmonary Anastomosis With Persistent Pleural Effusions.

Native pulmonary tract flow after a cavopulmonary anastomosis may promote pulmonary artery growth but can lead to undesirable consequences. We report the case of a 17-month child with prolonged pleural effusions after cavopulmonary anastomosis in whom a ventricular septal defect occluder device was placed in the native right ventricular outflow tract. (Level of Difficulty: Advanced.).

A Case of Trisomy 13 Mosaicism With Aortic Root Dilatation.

We present a 6-year-old female child, with prenatal diagnosis of trisomy 13 mosaicism and nondisjunction, a bicuspid aortic valve, and severe aortic root dilatation. This patient is only the third reported case of aortic dilatation in the setting of trisomy 13 mosaicism. (Level of Difficulty: Intermediate.).

Impact of right ventriculotomy for tetralogy of Fallot repair with a pulmonary valve-sparing procedure.

Objectives: The study objectives were to reconfirm the superiority of the pulmonary valve-sparing procedure versus the transannular patch procedure for repair of tetralogy of Fallot and to evaluate the influence of a right ventriculotomy in the pulmonary valve-sparing procedure. Methods: Between 1978 and 2003, 440 patients (aged <10 years) underwent tetralogy of Fallot repair. Of these patients, 242 (55.0%) underwent the transannular patch procedure, 106 (24.1%) underwent the pulmonary valve-sparing procedure without right ventriculotomy, and 92 (20.9%) underwent the pulmonary valve-sparing procedure with right ventriculotomy. End points focused on adverse events and included all-cause mortality, reoperation, catheter intervention, and symptomatic arrhythmias. To compare the outcomes of pulmonary valve sparing with and without right ventriculotomy, inverse probability weighting was applied to adjust for potential confounding factors. Results: The median follow-up period was 20.3 years (interquartile range, 10.7-27.6). In all cohorts, the pulmonary valve-sparing procedure was the independent factor that reduced adverse events after tetralogy of Fallot repair (hazard ratio, 0.47; 95% confidence interval, 0.23-0.94; P = .033). After weighting, there was no difference in overall survival or event-free survival in the pulmonary valve-sparing with and without right ventriculotomy group. However, the pulmonary valve-sparing with right ventriculotomy group exhibited a larger cardiothoracic ratio (beta: 6.01; 95% confidence interval, 2.36-9.66; P = .001), lower medication-free rate (odds ratio, 0.29; 95% confidence interval, 0.098-0.79; P = .019), and higher New York Heart Association functional classification (odds ratio, 2.99; 95% confidence interval, 1.36-6.80; P = .007) at the latest follow-up. Conclusions: Right ventriculotomy for tetralogy of Fallot repair with pulmonary valve-sparing did not increase major adverse events. However, negative impacts on current status cannot be ignored.

Persistent Patent Arterial Duct Rather Than a Ligament in the Setting of Diverticulum of Kommerell.

We report a rare case of persistent patent arterial duct (PDA) in the combination of diverticulum of Kommerell, aberrant left subclavian artery, and total anomalous pulmonary venous connection (TAPVC). Surgical correction of the defect was done by ligating and dividing the PDA and correcting the TAPVC. (Level of Difficulty: Intermediate.).

Pericardial effusion after congenital heart surgery.

Objective: Pericardial effusion after cardiac surgery remains an important cause of morbidity and mortality. We describe the risk factors of pericardial effusion after congenital heart surgery through analyzing data from a nationwide, multi-institutional registry. Methods: The Japan Congenital Cardiovascular Surgery Database, which reflects routine clinical care in Japan, was used for this retrospective cohort study. Multivariable regression analysis was done after univariable comparison of patients with pericardial effusion and no pericardial effusion. Results: The study enrolled 64,777 patients registered with the Japan Congenital Cardiovascular Surgery Database between 2008 and 2016; 909 of these had postoperative pericardial effusion (1.4%) and were analyzed along with 63,868 patients without pericardial effusion. Univariable analysis found no difference between the groups in terms of gender, early delivery, or preoperative mechanical ventilatory support. In the pericardial effusion group, cardiopulmonary bypass use was lower (58.4% vs 62.1%), whereas the cardiopulmonary bypass time (176.9 vs 139.9 minutes) and aortic crossclamp time (75.1 vs 62.2 minutes) were longer, and 30-day mortality was higher (4.1% vs 2.2%). Multivariable analysis identified trisomy 21 (odds ratio, 1.54), 22q.11 deletion (odds ratio, 2.17), first-time cardiac surgery (odds ratio, 2.01), and blood transfusion (odds ratio, 1.43) as independent risk factors of postoperative pericardial effusion. In contrast, neonates, infants, ventricular septal defect, atrial septal defect, tetralogy of Fallot repair, and arterial switch operation were correlated with a low risk of pericardial effusion development. Conclusions: The incidence of postoperative pericardial effusion in congenital cardiac surgery was 1.4%. Trisomy 21, 22q.11 deletion, first-time cardiac surgery, and blood transfusion were identified as the principal factors predicting the need for pericardial effusion drainage.

Transcatheter Aortic Valve Replacement in a Patient With Criss-Cross Heart.

This paper presents the first transcatheter management of severe aortic regurgitation in a 77-year-old woman with a criss-cross heart-an extremely rare and complex congenital heart disease. The procedure achieved an elimination of aortic regurgitation and resulted in a remarkable improvement of the patient's physical condition. (Level of Difficulty: Advanced.).

Postinfarction Ventricular Septal Rupture: Identification of the Failure Mechanism of a Percutaneous Closure Procedure.

Ventricular septal rupture (VSR) is a rare but highly lethal (∼60%) mechanical complication of myocardial infarction (MI). Although surgical repair has been the gold standard to correct the structural anomaly, percutaneous closure of the defect may represent a valuable therapeutic alternative, with the advantage of immediate shunt reduction to prevent further hemodynamic deterioration in patients with prohibitive surgical risk. Nonetheless, catheter-based VSR closure has faced certain drawbacks that have hampered its application. We describe a clinical case of postinfarction VSR treated with a percutaneous closure device and discuss the procedure's failure mechanism. (Level of Difficulty: Intermediate.).

Caudal regression syndrome: Postnatal radiological diagnosis with literature review of 83 cases.

Caudal regression syndrome (CRS) is a rare congenital disorder characterized by arrest of caudal spinal growth and associated with wide spectrum multisystemic anomalies. Herein, we presented a case of a newborn baby who did not pass meconium due to imperforated anus and was referred to the pediatric surgeon for urgent diverting loop colostomy. The conventional X-ray, abdominal ultrasound and abdominal pelvic magnetic resonance imaging (1.5 T) at 2-month-old age revealed right kidney agenesis, sacrococcygeal agenesis, vertebral bodies dysraphism and the spinal cord ends at D12-L1 with anterior and posterior bands of the terminating filaments. The diagnosis of CRS was confirmed. Through this case report, we hope to draw attention to this rare syndrome and the wide range of associated anomalies, also to consider this syndrome on the top of differential diagnosis list once the newborn has anorectal malformation mainly imperforated anus.

Accessory Left Ventricle: A Mistaken Case of Ventricular Septal Defect!

Physical examination is becoming a lost art. We describe a case of a patient who was referred to us with an initial diagnosis of ventricular septal defect. Discordance between imaging findings and the physical examination led to a diagnosis of an accessory left ventricle, a rare but benign congenital cardiac condition. (Level of Difficulty: Beginner.).

Transcatheter Closure of Left Ventricle to Coronary Sinus Fistula Post-MVR and Septal Myectomy.

This paper describes the case of a patient who developed refractory heart failure due to a fistula from the left ventricle to the coronary sinus that was unintentionally created after a surgical myectomy and mitral valve replacement. Advanced image guidance with a pre-procedure 3-dimensional physical model and intraprocedure echocardiography fusion facilitated transcatheter plugging of the shunt with symptom resolution. (Level of Difficulty: Advanced.).

Clinical update on the hybrid comprehensive stage II operation.

Objective: We previously described the hybrid comprehensive stage II operation as an alternate surgical procedure for a subset of patients with single ventricle congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the 4 patients who have undergone this procedure. Methods: After undergoing a hybrid approach to the stage I Norwood palliation, the hybrid comprehensive stage II procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection. With this approach, dissection of the distal arch and creation of a Damus-Kaye-Stansel anastomosis was avoided. A standard Fontan procedure was planned after the usual period of growth. Results: The first patient, who had trisomy 21 and elevated PA pressures, died postoperatively due to left PA thrombosis. The subsequent 3 patients survived the procedure and remain clinically well. All have required catheterizations for reintervention on their stented intrapulmonary baffles and ductal arches, and all have undergone successful completion of their Fontan procedures. Conclusions: The hybrid comprehensive stage II is a feasible, less complex alternative to the conventional comprehensive stage II operation in a subset of patients with single ventricle physiology. Early postoperative anticoagulation therapy to avoid PA thrombosis is recommended, and restenting of the ductal arch is anticipated. Although the long-term consequences of separate outflow tracts supplying the upper and lower body is unknown, the 3 surviving patients with this circulation are doing well with their Fontan circulation at midterm follow-up.

Homograft durability after correction of pulmonary atresia and ventricular septal defect with or without systemic pulmonary collateral arteries.

Background: Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature. Methods: This retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018. The primary interest was in homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure were analyzed with linear mixed-effects models. Results: The median duration of follow-up was 20 years. Of the 46 patients with SPCAs, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (interquartile range [IQR], 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCAs underwent successful complete correction at a median age of 1.6 years (IQR, 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. When a homograft was used during correction, freedom from homograft replacement after 20 years was comparable in the 2 groups (P = .925), at 32 ± 11% in the SPCA group and 32 ± 13% in the non-SPCA group. Indications for homograft replacement were isolated stenosis (n = 7; 46.7%), isolated regurgitation (n = 3; 20.0%), and mixed stenosis and regurgitation (n = 5; 33.3%) in the SPCA group and isolated stenosis (n = 8; 88.9%) and stenosis and regurgitation (n = 1; 11.1%) in the non-SPCA group. Peak homograft gradient was significantly (P = .0003) higher in patients without SPCA, with a comparable rate of progression in the 2 groups. However, the prevalence of severe pulmonary regurgitation (PR) was higher in patients with SPCAs, estimated at 35% at 10 years, compared with 15% in patients without SPCAs. Conclusions: Homografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, either with or without SPCAs, have similar limited durability. Repeated reintervention is common, and careful follow-up with attention to severe PR is warranted.

Use of Virtual Reality for Hybrid Closure of Multiple Ventricular Septal Defects.

A 28-month-old girl with multiple ventricular septal defects previously underwent surgical and transcatheter attempts at repair. Three-dimensional models were created from cardiac magnetic resonance-derived images. Viewing the models in virtual reality allowed the team to precisely locate the defects and decide on a hybrid transcatheter and surgical approach to ensure successful repair. (Level of Difficulty: Advanced.).

The prevalence of non-syndromic orofacial clefts and associated congenital heart diseases of a tertiary hospital in Riyadh, Saudi Arabia.

BACKGROUND: Orofacial clefts are considered one of the most common birth defects and are frequently associated with other malformations. Congenital heart disease is one of the most prevalent congenital malformation. OBJECTIVE: To investigate the prevalence of congenital heart diseases associated with non-syndromic orofacial clefts in the Saudi population. METHODS: Electronic files of non-syndromic orofacial cleft patients who visited the Oral and Maxillofacial Surgery Department in King Abdulaziz Medical City of Riyadh, Saudi Arabia from January 2015 to December 2018 were retrospectively reviewed. Data were recorded in an excel sheet and analyzed using SPSS via frequency tests. RESULTS: In the cleft children identified, the prevalence of non-syndromic orofacial clefts was (77%). Orofacial clefts showed a male predominance (62%). The most common orofacial phenotype was unilateral cleft lip and palate (34%). The prevalence of associated congenital malformations with orofacial clefts was (41%). The most prevalent congenital malformation was congenital heart disease (35%), mainly found in unilateral cleft lip and palate patients (33%). The prevalence of associated congenital heart disease with orofacial clefts was (19%). The most frequent type of congenital heart disease was atrial septal defect (37%). CONCLUSION: This study highlights the recognition of the associated congenital heart disease with non-syndromic orofacial cleft patients. Global screening protocols designed for newborns with non-syndromic orofacial cleft are needed to eliminate late diagnosis of critical congenital heart diseases which might present operative risks of anesthesia and/or surgical procedures.

Modified pulmonary artery banding: A novel strategy for balancing pulmonary blood flow with transposed great arteries.

OBJECTIVE: To study the outcomes of a novel modified pulmonary artery banding (mPAB) technique used for staged repair of a subset of patients with complex transposition physiology. METHODS: A total of 13 patients who underwent mPAB during their staged repair (biventricular repair [BVR], n = 6) or palliation (1-1/2 repair, n = 1; univentricular repair [UVR], n = 6) from 2004 to 2020 were studied retrospectively. A restrictive interposition graft was used to reconstruct the main PA between the pulmonary root and the distal pulmonary confluence, functioning as a mPAB. Twelve of the 13 patients (92.3%) underwent a concurrent arterial switch operation (ASO), of which 6 were palliative ASOs for 1-1/2 repair (n = 1) or UVR (n = 5). Patient weight and cardiac anatomy determined the size of interposition graft. RESULTS: The disease spectrum included dextro transposition of the great arteries (d-TGA) with multiple ventricular septal defects (VSDs) (n = 4), Taussig-Bing anomaly (n = 3), d-TGA with VSD and hypoplastic right ventricle (RV) (n = 3), double-inlet left ventricle with l-TGA (n = 2), and congenitally corrected TGA with double-outlet RV (n = 1). The Lecompte procedure was performed in 10 patients. Predischarge echocardiography revealed a band gradient of 61 mm Hg (interquartile range [IQR], 40-90 mm Hg) for BVR/1-1/2 ventricular repair (n = 7) and 49 mm Hg (IQR, 37-61 mm Hg) for UVR (n = 6). Survival was 100% at a median follow-up of 3.7 years (IQR, 2.6-4.0 years). CONCLUSIONS: The mPAB technique is effective and reproducible for staged BVR or UVR for patients with TGA. It effectively regulates pulmonary blood flow, may reduce neopulmonary root distortion, and eliminates complications associated with band migration in standard PAB.