Direct reimplantation technique via a right minithoracotomy for scimitar syndrome: a case report

Scimitar syndrome (SS) is a rare congenital cardiac anomaly defined by an anomalous right pulmonary vein draining of the right lung into the inferior vena cava. We describe a direct reimplantation technique and atrial septal defect closure using cardiopulmonary bypass via a right minithoracotomy on a 24 year-old female SS patient who had an accompanying sinus venous atrial septal defect.     

Left partial anomalous pulmonary venous connection found during a lobectomy for lung cancer: Report of a case

We report herein the case of a 68-year-old man in whom a partial anomalous pulmonary venous connection (PAPVC) was found during an operation for primary lung cancer. The preoperative clinical findings did not suggest a vascular shunt, and intraoperatively the anomalous vein was seen to drain only from the left upper lobe into the left innominate vein. The lower pulmonary vein connected normally, and there was no atrial septal defect nor any other anomalous condition. A left upper lobectomy with ligation of the anomalous connected vein was performed uneventfully. This type of PAPVC is extremely rare, and is especially noteworthy because there were no clinical signs.     

Atypical infantile form of scimitar syndrome with bronchomalacia

A male infant presenting with severe heart failure and respiratory distress was diagnosed with a hypoplastic right lung, scimitar syndrome with pulmonary sequestration and multiple anomalous systemic arteries, left bronchomalacia, a large atrial septal defect and coarctation of the aorta. The infant underwent a successful combined treatment of surgical and transcatheter intervention, including coil embolization and endobronchial stenting.     

Transcatheter treatment of Lutembacher's syndrome

Lutembacher's syndrome is a rare combination of atrial septal defect (ASD) and rheumatic mitral stenosis. Traditionally, this condition is treated surgically. We present a case of Lutembacher's syndrome that was successfully treated with percutaneous transcatheter mitral commissurotomy (PTMC) using the Inoue balloon. Closure of ASD was successfully achieved with an Amplatzer atrial septal occluder under transthoracic echocardiogram (TTE) guidance without general anesthesia.     

A case of large congenital right ventricular outflow aneurysm]

A rare case of large congenital right ventricular outflow aneurysm is reported. A 5-year-old girl had been diagnosed as having atrial septal defect and pulmonary valvular stenosis. A large cavity in front of right ventricular outflow was noticed by her follow-up echocardiography. The non-contracting cavity was confirmed as an aneurysm originating from right ventricular outflow tract by angiocardiogram. Surgical resection was performed successfully with concomitant atrial septal defect closure and pulmonary valvular commissurotomy. Histological examination revealed that the wall of the ventricular outflow aneurysm consisted of dense fibrous tissue, contained no myocardium, and the cavity was lined with endocardium. It is reported that rupture of congenital ventricular aneurysm may occur even if there is no symptom. Surgical resection will be recommended especially when increasing in size is diagnosed.     

Bispectral index in a 3-year old undergoing deep hypothermia and circulatory arrest

We report a 3-year-old girl who presented with Scimitar syndrome and underwent hypothermic circulatory arrest for correction of anomalous pulmonary veins and an atrial septal defect. In this case the Bispectral Index (BIS) correlated significantly with the gradual onset of hypothermia and circulatory arrest. However, BIS remained low during the rewarming phase of cardiopulmonary bypass, in spite of adequate pump flows and stable haemodynamics. We postulate that this significant lag in BIS during the rewarming phase of deep hypothermic circulatory arrest may represent neuronal bewilderment or perhaps stunning, and differs from previous studies that show significant increase in BIS during rewarming from mild hypothermia.     

Successful liver transplantation in an adult residual atrial septal defect and left-to-right shunt

A case of orthotopic liver transplantation performed in an adult, presenting postviral C cirrhosis and a residual atrial septal defect, is reported. This defect was responsible for a left-to-right shunt and moderate systolic pulmonary hypertension. Liver transplantation was done using an inferior vena cava preservation technique with a latero-lateral cavo-caval anastomosis and without veno-venous bypass in order to minimally interfere with the haemodynamic condition. This case report shows that adaptation of surgical technique may allow successful liver transplantation in cases of haemodynamically significant atrial septal defects.     

Reduction in recalcitrant pulmonary hypertension after operation for atrial septal defect

We present the case of a patient with atrial septal defect and severe pulmonary hypertension with pulmonary artery peak pressure greater than 110 mm Hg. Open lung biopsy was done prior to the corrective operation, and pathological findings in the small pulmonary arteries included "musculoelastosis" and complete occlusion of 70% of these small arteries and arterioles. The atrial septal defect was closed, and long-term oral prostacyclin therapy was initiated. Pulmonary artery peak pressure decreased to 65 mm Hg 2 years after the operation. This case demonstrates that in a patient with 70% complete occlusion of small pulmonary arteries and arterioles resulting from "musculoelastosis," not only is surgical intervention possible but also pulmonary artery pressure decreases in the long term after operation.     

Cyanosis in atrial septal defect without pulmonary hypertension: a case of platypnea-orthodeoxya syndrome

Cyanosis in atrial septal defect typically occurs when pulmonary hypertension develops. Platypnea-orthodeoxya is an uncommon syndrome, still under debate, characterized by breathlessness and arterial oxygen desaturation exacerbated in the upright position. An interatrial communication is a common finding in this syndrome, but the absence of a right to left pressure gradient complicates the physiopathological picture. To explain the right to left shunt, it is generally advocated a concomitant condition that alternates the sterical relationship between inferior vena cava orifice and the atrial septal defect. A case of a 58-year-old male with platypnea-orthodeoxya syndrome related to a fenestrated redundant interatrial septum without any additional pathologic condition is reported. Possibly, this isolated anatomical abnormality could lead to a right to left shunt in the absence of other coexisting predisposing factors. It is reasonable to hypothesize the septum secundum bulging like a 'spinnaker' into the right atrium, so that it deviates the inferior vena cava venous blood towards the left atrium. Echocardiographic evaluation is mandatory to achieve a correct diagnosis and to decide the therapeutic strategy.     

静脉窦型房间隔缺损的外科治疗

目的 总结静脉窦型房间隔缺损（SVASD）外科治疗的方法和经验。方法 32例SVASD患者均伴有右侧部分或全部的肺静脉异位引流。上腔SVASD25例，其中22例采用双片心包补片修补，3例将异位引流的肺静脉改道、上腔静脉与右心耳吻合（Warden法）进行手术纠治。7例下腔SVASD患者中，3例为弯刀综合征，关闭房间隔缺损（ASD）的同时将异位引流的肺静脉离断后与左心房后壁吻合；其余4例采用单片心包补片修补ASD。结果 手术效果满意，无手术死亡。随访28例，心电图检查均为窦性心律；心脏超声心动图检查提示：心内无残余分流、右肺静脉及腔静脉回流无梗阻。结论 SVASD特殊的解剖结构决定了其多伴右侧肺静脉异位引流，纠治上腔SVASD时应注意避免损伤窦房结，防止上腔静脉回流梗阻；但弯刀综合征多伴右肺发育不良，心功能情况还须长期随访。     

Incidental partial anomalous pulmonary venous connection in left lung cancer

We present the case of a 79-year-old woman with partial anomalous pulmonary venous connection (PAPVC), discovered incidentally during upper left division segmentectomy for primary lung cancer. The left superior pulmonary vein originated from the hilum of the upper left lobe and flowed into the left brachiocephalic vein. The left inferior pulmonary vein was connected normally, and neither atrial septal defect nor other anomalous condition was present. Upper left lobectomy with ligation of the anomalous connected vein was performed, as the lingual segment was anatomically difficult to retain. Although this type of PAPVC is extremely rare, it is advisable to exercise caution when performing lung resection with the potential for PAPVC in mind.     

Incidental partial anomalous pulmonary venous connection in left lung cancer.

We present the case of a 79-year-old woman with partial anomalous pulmonary venous connection (PAPVC), discovered incidentally during upper left division segmentectomy for primary lung cancer. The left superior pulmonary vein originated from the hilum of the upper left lobe and flowed into the left brachiocephalic vein. The left inferior pulmonary vein was connected normally, and neither atrial septal defect nor other anomalous condition was present. Upper left lobectomy with ligation of the anomalous connected vein was performed, as the lingual segment was anatomically difficult to retain. Although this type of PAPVC is extremely rare, it is advisable to exercise caution when performing lung resection with the potential for PAPVC in mind.     

A surgical case of atrial septal defect with myotonic dystrophy

A case report is presented of a 16-year-old woman with myotonic dystrophy, who required open-heart surgery for an atrial septal defect. We successfully repaired an atrial septal defect using normothermic cardiopulmonary bypass and electrically maintained ventricular fibrillation. Anesthesia using fentanyl, vecronium and isoflurane was uneventful and there were no postoperative complications. Anesthetic agents must be selected with care, and ventilation requires meticulous attention.     

Cardiac rhabdomyomas with atrial septal defect and tricuspid insufficiency: A case report

Primary cardiac tumors are very rare and generally benign. The most common type, cardiac rhabdomyoma, comprises 45% to 75% of primary cardiac tumors. Cardiac rhabdomyoma is a rare benign tumor that commonly presents with tuberous sclerosis. We present a case of an infant with multifocal cardiac rhabdomyomas with an atrial septal defect and tricuspid insufficiency and no sign of tuberous sclerosis. She was successfully treated with an operation, the treatment plan included mass resection, tricuspid annuloplasty, and closure of the patent foramen ovale. The right atrial lesion was resected entirely, while the lobulated lesion in the right ventricle was resected as two pieces. There was no evidence of recurrence 1 year after the surgery.     

Pediatric lung transplantation for pulmonary hypertension and congenital heart disease.

Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.     

Congenital tricuspid stenosis treated by a palliative open operation. Report of a case.

The case is presented of a patient with congenital tricuspid stenosis who was treated successfully by a palliative open operation. The preoperative diagnosis was tricuspid atresia. At operation, however, congenital tricuspid stenosis was detected and managed by commissurotomy.. The postoperative course was very good. Later, the associated ventricular septal defect (VSD), pulmonary annular stenosis, and probably the atrial septal defect (ASD) will be corrected. We emphasize the importance of diagnosing congenital tricuspid stenosis correctly, because of the possibility of operating upon the valve itself.     

Management of a young patient with dextrocardia,atrial septal defect,and Eisenmenger syndrome with venous‐venous extracorporeal membrane oxygenation and heart‐lung transplantation

Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which ultimately led to decompensated end‐stage lung disease and heart‐lung transplant. Venous‐venous extracorporeal membrane oxygenation was an important strategy to bridge the patient until donor organs became available. Transplantation of a heart‐lung block allowed for the treatment of the patient's underlying congenital heart defect, anatomic reversal of dextrocardia with appropriate venous and arterial connections, and management of pulmonary damage from pulmonary hypertension.     

A case of tricuspid pouch associated with ventricular septal defect and functional left ventricular-right atrial communication]

We reported a 60-year-old woman with tricuspid pouch associated with ventricular septal defect and subsequent left ventricular-right atrial communication. Preoperative angiographic findings suggested the presence of membranous septal aneurysm, ventricular and atrial septal defects, and tricuspid insufficiency. However, at operation, besides perimembranous inlet type ventricular septal defect, a pouch, 1.5 cm in diameter was found in the adjacent part to the septal leaflet within the anterior one of the tricuspid valve. The ventricular septal defect was closed with a patch. From these findings, it is speculated that the tricuspid pouch was formed by the effect of jet stream through the ventricular septal defect.     

Postoperative management of postpneumonectomy pulmonary edema in a patient with ostium secundum atrial septal defect

We report the case of a 55-year-old woman who underwent right pneumonectomy for invasive squamous cell carcinoma. At 48 hours after surgery she developed severe dyspnea and hypoxemia that required reintubation and a progressively higher inspired oxygen fraction. A radiograph demonstrated pulmonary edema; echocardiography revealed an ostium secundum atrial septal defect with increased flow to the lung and severe pulmonary hypertension. Emergency percutaneous closure of this defect was carried out. This case report describes the management and treatment of postpneumonectomy pulmonary edema and atrial septal defect, as well as the circumstances that favor their development. We recommend that cardiac defects be investigated and possible treatment be considered prior to pneumonectomy.     

Case report of scimitar syndrome in infancy: intracardiac repair after coil embolization of systemic arterial supply to sequestered lung

A 10-month-old girl was diagnosed with atrial septal defect and pulmonary sequestration and scimitar syndrome. Cardiac catheterization revealed severe pulmonary hypertension, with systemic pulmonary artery pressure 97 mmHg, pulmonary resistance 10.3 U, and Qp/Qs 0.45. Systemic pulmonary artery pressure was decreased from 108 to 75 mmHg by oxygenation. We performed coil embolization of the systemic artery and repair of the partial anomalous pulmonary venous return, division and reimplantation of the anomalous vein into the right atrium, and a patch was inserted to redirect flow to the left atrium. The severe pulmonary hypertension was improved and echocardiography showed systemic pulmonary artery pressure of 45 mmHg. The postoperative hemodynamic and respiratory condition was satisfactory.