Factors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect where pulmonary venous plexus fails to connect with the left atrium (LA). Surgical repair is the primary treatment for TAPVC, but factors influencing outcomes are not fully understood. This study investigates the early outcomes of surgical repair for TAPVC and associated factors. A retrospective cohort analysis was conducted on TAPVC patients who underwent surgical repair between 2012 and 2022. Data were collected from medical records and supplemented with phone call validation. Demographic characteristics, surgical data, diagnostic tests, and outcomes were analyzed. Statistical analysis included chi-square, t-tests, and multivariate logistic regression using SPSS. A total of 88 patients underwent surgical repair for TAPVC, resulting in a mortality rate of 21.6%. Weight and bypass time were significantly associated with patient survival. Female patients had a higher likelihood of death. The anatomic type did not significantly influence mortality. Patients with pulmonary venous obstruction (PVO) experienced a higher mortality rate. Notably, ligation of the vertical vein in supracardiac and infracardiac types was associated with lower mortality. In conclusion, our study identifies several key factors contributing to higher mortality rates following TAPVC surgery, including low weight, female gender, prolonged bypass time, and preoperative vein obstruction. Highlighting the significance of surgical technique, particularly the sutureless approach, we advocate for its meticulous consideration to achieve improved outcomes. Furthermore, our findings indicate a potential decrease in mortality associated with vertical vein ligation, which may mitigate the risk of post-repair heart failure. We suggest further rigorous studies to gain comprehensive insights into TAPVC surgical interventions.

A Rare Combination of Cardiac Hydatid Cyst and Pulmonary Hydatidosis in a Child.

Hydatid disease is a common health problem in the sheep-farming countries of the Mediterranean, caused by infection with Echinococcus granulosus. The common sites are the liver and lungs. Cardiac hydatidosis is rare. Herein, we report a case of interventricular septum hydatid cyst with lung involvement in a four-year-old child. Cardiac hydatid cysts should be resected surgically even in asymptomatic patients since delayed diagnosis and treatment can lead to fatal outcomes. In spite of its rarity, the combination of cardiac and pulmonary hydatidosis can be managed with a single operation.

Unobstructed infracardiac total anomalous pulmonary venous connection in a 7-month-old infant: A rare case report.

Key Clinical Message: This case underscores the importance of early detection and treatment for total anomalous pulmonary venous connection, a rare congenital heart condition, through comprehensive newborn physical exams and prompt specialist referrals. Abstract: Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease that is typically diagnosed in neonates. TAPVC has four subtypes, with the infracardiac type at risk of obstruction. TAPVC is usually diagnosed in newborns but can occur in other age groups. In this case, a 7-month-old male with recurrent cyanotic episodes was diagnosed with TAPVC of the infracardiac type using computed tomography angiography. The patient underwent successful surgical repair with a favorable postoperative course and was discharged in stable condition, and further follow-up was not possible beyond 2 months. This case emphasizes the importance of early recognition and management of this condition to prevent the progression of subsequent complications.

Misdiagnosis of persistent left superior vena cava with unroofed coronary sinus as a coronary sinus-type atrial septal defect.

Key Clinical Message: Awareness of persistent left superior vena cava (PLSVC) with unroofed coronary sinus is crucial. Pre- and perioperative evaluation of this association is necessary for surgical plan. Creating an intra-atrial tunnel to divert LSVC to right atrium without obstructing the mitral valve or the pulmonary veins is the safe surgical approach. Abstract: Unroofed coronary sinus syndrome is a rare congenital heart defect representing less than 1% of all atrial septal defect (ASD) types, and may be associated with persistent left superior vena cava (PLSVC) which may be missed during preoperative diagnosis. Herein, we present a case of a 2-year-old patient who underwent an operation for repair of a coronary sinus-type ASD; however, PLSVC was detected intraoperatively. An antra-atrial tunnel has created to divert the flow of PLSVC into the right atrium along with the repair of the ASD.

Konno-Rastan Operation in a Patient With Dextrocardia and Situs Inversus.

The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.

Long-term outcomes of surgical repair of isolated coarctation of the aorta in different age groups.

BACKGROUND: Coarctation of the aorta (CoA) is one of the most common congenital heart defects (5-8% of all CHD). Treatment of native CoA may be accomplished surgically, or through an interventional approach. Surgical repair of CoA remains an important option for treatment of aortic coarctation during childhood, although it is mostly performed in neonates and young infants. OBJECTIVES: In this retrospective study, we sought to share the long-term outcomes of different surgical techniques for repair of coarctation of the aorta in different age groups. MATERIALS AND METHODS: This is a retrospective single-center clinical study that included 228 consecutive patients (age: 1 day- 41years) in whom surgical repair of isolated native coarctation of the aorta was performed with different surgical techniques. RESULTS: Immediate results were excellent; however, the mortality rate were higher in the infants. Complications rate and incidence of recoarctation, both were comparable between different age groups and different surgical techniques. CONCLUSIONS: Surgical repair of CoA remains an important option for treatment of aortic coarctation in different age groups with low morbidity and mortality. We did not find any significant difference between different surgical techniques regarding the development of recoarctation.

Surgical repair of an obstructed mixed-type total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital anomaly and has four anatomical subtypes of which the mixed type represents diagnostic and therapeutic challenge. When associated with obstruction, however, urgent surgical repair is needed. Herein, we present a rare case of obstructed mixed type total anomalous pulmonary venous connection with successful surgical repair.

Repair of double orifice mitral valve with an atrioventricular septal defect in a girl with Ellis-Van Creveld syndrome.

Ellis-van Creveld syndrome is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. The four principal manifestations are chondrodysplasia, polydactyly, ectodermal dysplasia, and congenital heart defects. We describe the case of a 7-year-old girl with Ellis-van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications.

Surgical repair of left-sided partial anomalous pulmonary venous connection associated with situs inversus totalis.

The recognition of situs inversus is of great importance to avoid possible surgical mishaps that may result from the failure to recognize reversed anatomy or an atypical history. The repair of associated PAPVC with situs inversus totalis needs special attention during surgery due to the reversed anatomy.ss.

Surgical excision of a cardiac hydatid cyst from the right ventricle in a child.

Reporting the rare cardiac localization of hydatid disease in the right ventricle of a child, and underlining the importance of early diagnosis and treatment to avoid severe and life-threatening complications.

Surgical repair of a giant congenital left atrial aneurysm presenting with respiratory distress in a neonate.

The presentation of congenital left atrial aneurysm is extremely rare in neonates. The neonate may suffer from severe respiratory distress symptoms, and by then, early surgical management is lifesaving.

Coronary artery bypass grafting in a 12-year-old girl with familial hypercholesterolemia.

As CABG operation is indicated in children with familial hypercholesterolemia, it seems that bilateral internal thoracic arteries are the preferred grafts, taking into consideration its superiority over venous graft regarding patency and growth.

Surgical repair of an isolated left-sided partial anomalous pulmonary venous connection in an 18-month-old child.

The isolated left-sided partial anomalous pulmonary venous connection is a rare congenital anomaly. Only 3% of cases have been reported with drainage from the whole left lung into the left innominate vein. This anomaly is often asymptomatic and may go undetected (particularly in non-referral centers) until adulthood when symptoms begin to manifest. Transthoracic echocardiography is the main diagnostic tool; however, computed tomography angiography, and magnetic resonance imaging are widely used nowadays. Herein, we present the case of an 18-month-old girl with the diagnosis of isolated left-sided partial anomalous pulmonary venous connection as the left pulmonary veins were draining into the left innominate vein via a vertical vein. The patient underwent surgical repair by anastomosing the vertical vein to the left atrial appendage using cardiopulmonary bypass and cardiac arrest. The patient was discharged on the fifth day without complications. The precise follow-up for 12 months showed improved clinical symptoms, and the successively performed TTE confirmed the excellent result of the operation.

A Case of Ewing Sarcoma Presenting With Cardiac Metastasis.

Ewing sarcoma is the second most prevalent primary malignant bone tumor. Metastases from Ewing sarcoma to the heart are very rare. Herein, we present a case of undiagnosed Ewing sarcoma in a 12-year-old boy who presented with metastasis to the left atrium. The mass was prolapsing across the mitral valve. In view of the potential risk of embolization, urgent cardiac surgery was performed before the establishment of a definitive diagnosis. Histopathology was consistent with Ewing sarcoma. Subsequent magnetic resonance imaging revealed a mass in his left fibula. The patient was referred to the oncology center and received chemotherapy. One year later, he is alive with no evidence of recurrent cardiac involvement.

Mid-term outcomes of mechanical pulmonary valve replacement: a single-institutional experience of 396 patients.

OBJECTIVES: Previous small-sized studies have demonstrated the safety and efficacy of mechanical pulmonary valve replacement (mPVR) in patients with congenital heart disease; however, the predictors of major complications and reoperation remained unclear. METHODS: In a retrospective study, we reported the mid-term outcomes of a large-scaled series of patients, 396 patients, with congenital heart diseases who underwent mPVR in a single institution. RESULTS: The patients' mean age at mPVR was 24.3 ± 9 years (4-58 years). Most patients (84.3%) underwent tetralogy of Fallot total correction. The median of follow-up was 36 months (24-49 months). Prosthetic valve malfunction caused by thrombosis or pannus formation developed in 12.1% of patients during follow-up period. Reoperation was performed in 7 cases with pannus formation and 6 cases with mechanical valve thrombosis. Freedom from reoperation at 1, 5, and 10 years was 99%, 97%, and 96%, respectively. Neither early nor mid-term mortalities were detected. Cox regression models showed that male gender and smaller valve size increased the risk of prosthetic valve failure. The age at mPVR, interval between congenital heart defect repair and mPVR, and concomitant procedures predicted reoperation. In multivariate analysis, younger age and the interval between first operation and mPVR predicted reoperation either. CONCLUSIONS: The success rate of mPVR is excellent in mid-term follow-up. Younger age, longer interval between the repair of congenital defect and mPVR, and cooperation increased reoperation risk. However, strict adherence to life-long anticoagulation regimen and patient selection are of great importance for the implementation of mPVR.

Intraoperative diagnosis of a quadricuspid aortic valve.

Quadricuspid aortic valve is a rare anomaly, and most patients require surgery for aortic regurgitation in the 5th or 6th decades of life; only a few cases of aortic valve repair in childhood have been reported. A 3-year-old boy was scheduled for ventricular septal defect closure and aortic valve repair. Quadricuspid aortic valve was an incidental finding at operation; it was repaired by joining the left anterior and right anterior cusps. At the 9-month follow-up, the patient had no more than mild aortic regurgitation. We emphasize the importance of detecting this anomaly, especially in children with aortic valve regurgitation.

The Outcomes of Superior Cavopulmonary Connection Operation: a Single Center Experience.

INTRODUCTION: The superior cavopulmonary connection operation is one of the stages of the palliative surgical management for patients with functionally single ventricle. After surviving this stage, the patients are potential candidates for the final palliative procedure: the Fontan operation. OBJECTIVES: This study aimed to analyze the outcomes of superior cavopulmonary connection operations in our center and to identify factors affecting the survival and the progression to Fontan stage. METHODS: The outcomes of 161 patients were retrospectively analyzed after undergoing superior cavopulmonary connection operation in our center between 2005 and 2015. RESULTS: The early mortality rate was 2.5%. Five (3.1%) patients underwent takedown of the superior cavopulmonary connection. The rate of exclusion from the Fontan stage was 8.3%. Statistical analysis revealed that elevated mean pulmonary artery pressure preoperatively and the prior palliation with pulmonary artery banding were risk factors for both early mortality and takedown; however, the age, the morphology of the single ventricle and the type of operation were not considered risk factors. CONCLUSION: The superior cavopulmonary connection operation can be performed with low rate mortality and morbidity; however, the elevated mean pulmonary artery pressure preoperatively and the prior pulmonary artery banding are associated with poor outcomes.

The outcomes of superior cavopulmonary connection operation: a single center experience

Abstract Introduction: The superior cavopulmonary connection operation is one of the stages of the palliative surgical management for patients with functionally single ventricle. After surviving this stage, the patients are potential candidates for the final palliative procedure: the Fontan operation. Objectives: This study aimed to analyze the outcomes of superior cavopulmonary connection operations in our center and to identify factors affecting the survival and the progression to Fontan stage. Methods: The outcomes of 161 patients were retrospectively analyzed after undergoing superior cavopulmonary connection operation in our center between 2005 and 2015. Results: The early mortality rate was 2.5%. Five (3.1%) patients underwent takedown of the superior cavopulmonary connection. The rate of exclusion from the Fontan stage was 8.3%. Statistical analysis revealed that elevated mean pulmonary artery pressure preoperatively and the prior palliation with pulmonary artery banding were risk factors for both early mortality and takedown; however, the age, the morphology of the single ventricle and the type of operation were not considered risk factors. Conclusion: The superior cavopulmonary connection operation can be performed with low rate mortality and morbidity; however, the elevated mean pulmonary artery pressure preoperatively and the prior pulmonary artery banding are associated with poor outcomes.

Different Presentation of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Adults: Case Reports.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac malformation. We report three cases of ALCAPA who survived to adulthood. The first case was a 51-year-old woman who complained of typical chest pain that was diagnosed with ALCAPA using cardiac catheterization and coronary computed tomographic angiography (CTA). The second case was a 30-year-old woman with a history of surgery for atrial septal defect at 10 years old who presented with progressive exertional dyspnea. Cardiac catheterization confirmed the diagnosis of ALCAPA. The third case was a 19-year-old man who was brought to our clinic due to aborted sudden cardiac death on the previous day. Cardiac catheterization and coronary CTA confirmed the diagnosis. They underwent the closure of orifice of the anomalous left coronary artery and grafting the left anterior descending artery concomitantly with mitral valve repair. All patients were followed up during a mean of 8.7 months and they were asymptomatic.