Double-outlet left atrium with intact ventricular septum

The clinical course and surgical repair of double-outlet left atrium with intact ventricular septum in a 13-year-old girl are presented. The only outlet of the right atrium was a secundum atrial septal defect, and the left atrium drained into both ventricles through two atrioventricular valves. To our knowledge, there has been only one other published report of repair of double-outlet left atrium.     

Simultaneous clinical manifestation of subependymoma of the fourth ventricle in identical twins. Case report

At the age of 22 years, identical twin brothers simultaneously developed symptoms of intracranial pressure. Radiological investigation revealed cerebellar midline tumors with occlusive hydrocephalus of the third and lateral ventricles. At operation, subependymomas with identical histological features were found in the fourth ventricle in both twins. This is the first report of subependymomas in identical twins. The clinical data suggest that this tumor type is of maldevelopmental origin.     

胸腹联体双胎婴儿分离术的体会

目的　通过胸腹联体双胎婴儿的分离手术提供联体婴儿外科诊疗方法。方法　术前经X片、CT、MRI和B超等确诊,该联体婴共用一副肝脏、心包、膈肌、胸骨中段及5～10肋相连,胸膜紧贴。有2个心脏（A婴心脏突入B婴心包腔,并伴有房室间隔缺损）、两副消化道和胆道。经积极术前准备,在营养和肝功能改善后,于生后45天在多科协作下成功地进行联体分离手术。结果　术中见腹腔中有各自的消化道,共同肝相连面积约7 cm×6 cm,用电刀切开。胸腔中,见两婴的心脏共用一个心包,也予以切开、用人造涤沦片修补缺损。胸骨、肋骨也分别切开。A婴的胸腔用医用硅胶板做成支架,予以扩大。术后二婴皆存活。结论　联体婴儿术前正确评估,正确选用麻醉和手术方法,术后加强监护是手术成功的关键。     

Parapagus conjoined twins with unilateral mesenteric venous outflow

This case presents a complication of attempted separation of parapagus conjoined twins, related to loss of an intact mesenteric-portal venous axis. Despite known lack of a superior mesenteric artery in the right twin, initial evaluation in the operating room suggested that separation was possible. After hepatic division, however, it became apparent that the mesenteric drainage was not separable; and the operation was aborted. Subsequently, significant growth failure and hypoglycemia were noted in the right twin. The situation was corrected by creating a shunt to reinstitute mesenteric flow to the right twin's liver and separating the twin's mesenteric drainage. One year postoperatively, both twins are independently nourishing themselves and have been free from hospitalization with stable glucoses.     

Temporoparietal craniopagus. Case report and review of the literature

A case of craniopagus twins joined in the temporoparietal area is presented, along with a review of the literature on craniopagus. A large area of brain was shared between the neurologically normal infants, with defects in the scalp, skull, and dura. The twins were separated in a three-step procedure. First, areas of shared brain were divided and separated with silicone sheets. The second procedure consisted of the insertion of scalp expanders to allow primary skin closure. In the third procedure complete separation was performed which was complicated by severe hypotension in one infant that was due to dural sinus hemorrhage. Cerebrospinal fluid leak was the most difficult problem encountered in the postoperative period; this was treated with lumboperitoneal and ventriculoperitoneal shunts. After 2 years, one twin is neurologically normal; the other is severely developmentally delayed, possibly related to the severe hypotension experienced during the third procedure. A review of the literature on craniopagus is presented. Analysis of data in the literature suggests that the area involved in the craniopagus as well as the venous connections are closely related to survival following separation of craniopagus twins.     

Successful early separation of a premature xipho-omphalopagus conjoined twins: a case report

Premature xiphoomphalopagus conjoined twins were successfully separated at 7 days of age. The total body weight of the twins before separation was 3502 g. One twin had persistent patent ductus arteriosus and signs of cardiac failure at 4 days of age. This was managed with indomethacin. Thirty-six hours later the second twin became anuric, necessitating early emergency separation. The twins were joined from the lower sternum to the infraumbilical area. The liver was fused, but there was no major vascular connection. There were no other major anomalies. The babies stood the procedure well and were healthy at follow-up 8 months after separation.     

Differential energy metabolism in conjoined twins.

BACKGROUND/PURPOSE: Conjoined twins often have different body composition and growth rate before separation. This may be because of differences in energy metabolism. The aim of this study was to investigate the energy expenditure, body composition, and calorie intake of thoracopagus conjoined twins with shared hepatic circulation but separate gastrointestinal tracts. METHODS: The twins were studied at two periods: (1) before separation (age, 73 days) and (2) after separation (age, 97 days). Calorie intake over the study periods was carefully documented. Respiratory gas exchange was measured by computerized indirect calorimetry. The postseparation weight ratio of twin A to twin B was used to approximate the preseparation weights. Body composition (total body fat) was calculated from skinfold thickness and anthropometric measurements. RESULTS: The body composition of the twins was different: body weight and total body fat were higher in twin B. Resting energy expenditure and calorie intake were markedly different between the conjoined twins before separation. In both twins, the energy expenditure increased after separation. After separation, the resting energy expenditure of the twins was similar. CONCLUSIONS: This study illustrates the difference in energy metabolism in a set of thoracopagus conjoined twins. The authors speculate that twin A was supplying nutrients to twin B resulting in increased energy expenditure before separation. This would explain the lower calorie intake and higher fat mass of twin B.     

Twenty-three-year follow-up of separated ischiopagus tetrapus conjoined twins.

This paper presents a 23-year follow-up of the separation of ischiopagus tetrapus conjoined twins reported in Annals of Surgery in December 1966. One twin died of septicemia at age 2 years after bilateral pelvic osteotomies for the treatment of her marked pelvic diastasis. The surviving twin has done reasonably well, and her most significant problem is related to her musculoskeletal system. She has an increasing T7-10, L-1 apex right congenital scoliosis with wedged vertebra at T-10, as well as marked pubic diastasis and bilateral subluxation of her hips. This has resulted in a somewhat aberrant physical appearance and a "waddling" gait. Her colostomy functions well and she has normal renal and bladder function. This patient's history illustrates that many problems remain after successful separation of conjoined twins. However these problems are manageable and do not preclude the possibility that such a patient may be a productive member of society.     

Anaesthetic managements for conjoined twins with complex cardiac anomalies

Purpose

To describe the perioperative assessment and anaesthetic management for surgical separation of three sets of conjoined twins with complex cardiac anomalies threatened with arterial desaturation and haemodynamic instability.

Clinical features

Three sets of conjoined twins, one omphaloischiopagus, one omphalopagus, and one thoraco-omphalopagus, were considered for separation during the perinatal or infantile period. Preoperative functional evaluation including continuous pulse oximetry, capnography, and cardiac electrophysiological studies were considered to be as important as anatomical evaluation of the cardiac anomalies and cross-circulation by angiography in assessing the feasibility of surgical separation. Ipsilateral infusion of prostaglandin E₁ and phenylephrine were applied to the cyanotic and healthy twins respectively, to restore arterial oxygenation intraoperatively and to avoid profound hypoxaemia.

Conclusion

Surgical separation and anaesthesia should be well planned and rehearsed before clinical deterioration of the weaker twin. Aggressive pharmacological intervention and understanding of the cross-circulation pathophysiology is necessary to manage critical situations during surgical separation and in the postoperative period.     

Emergency separation of conjoined twins.

Female omphalopagus conjoined twins were successfully delivered vaginally and required emergency surgical separation shortly after birth for gastroschisis. Shared tissue included conjoined bowel; one twin also had a complex cloacal abnormality and patent urachus. A 2-year follow-up is presented. A review of the relevant literature confirms that this is the first example of gastroschisis conjoining omphalopagus twins.     

Surgical therapy of paroxysmal atrial fibrillation with the "corridor" operation.

Patients with paroxysmal atrial fibrillation may be extremely disabled despite medical therapy. Based on recent concepts of atrial fibrillation, a surgical open heart procedure was designed to isolate a "corridor" from the right and the left atrium. The corridor consists of the sinus node area, the atrioventricular nodal junction, and the connecting right atrial mass, small enough to prevent atrial fibrillation. Between 1987 and 1990, 20 patients with severely disabling symptoms due to frequent paroxysmal atrial fibrillation underwent the corridor operation, with permanent success in 16 patients. In 8 patients, left atrium to corridor conduction reappeared shortly after the procedure. Reoperation was performed in these patients without extracorporeal circulation. The site of persistent conduction between the left atrium and the corridor could consistently be localized adjacent to the coronary sinus. Nevertheless, reoperation failed to isolate permanently the corridor in 4 patients. During a mean follow-up of 20 months, atrial fibrillation dominating the ventricles was never observed nor inducible in the corridor in the 16 patients with a successful operation. In all cured patients, sinus node function remained undisturbed. Paroxysmal atrial flutter inside the corridor arose in 1 patient and a paroxysmal focal tachycardia in another. All 16 cured patients experienced a clear improvement in quality of life. Refinement of the surgical technique to obtain persistent isolation between the left atrium and the corridor is needed. These results demonstrate that the concept of the corridor operation is sound and justify its use in the treatment of drug-refractory paroxysmal atrial fibrillation.     

Eleven-year experience in diagnosis and surgical therapy of right atrial masses

BACKGROUND: Tumors arising from the right atrium are quite rare, and require special care during differential diagnosis for their management. A review of surgical experience with right atrial tumors in 11 patients from our institution has been presented in this article. METHODS: Eleven cases, operated for a tumor mass in the right atrium in our institution between January 1993 and December 2004, were retrospectively reviewed for their clinical presentation, diagnostic workup, method of surgical procedure, and histopathologic findings. Electrocardiogram, transthoracic, and transesophageal echocardiography, computerized tomography, and nuclear magnetic resonance imaging were available for all patients during the diagnostic evaluation. Surgical procedure notes, photos, and file recordings were reviewed when available. The surgeons were also interviewed when necessary. RESULTS: Right atrial tumors were diagnosed in 11 patients (6 males and 5 females). The average age of the patients was 34 +/- 11 years (ranging between 21 and 65 years). The histopathological examination of the surgically removed specimen revealed a benign tumor in eight patients (73%), and a malignant process in three (23%). In eight patients with a benign tumor, atrial myxoma was the leading cause in half of the cases. Hydatid cyst (n = 2), lipoma (n = 1), and right atrial thrombus (n = 1) were detected in the remaining four patients. One patient died of heart failure after surgery. The diameters of the excised masses were 2 +/- 0.5 cm versus 7 +/- 1 cm. CONCLUSIONS: Tumors of the right atrium are rarely seen, and necessitate a unique attention during the process of diagnosis and surgical treatment. We present our surgical experience of 11 patients with right atrial mass. The differentiation of the right atrial tumors with the diagnostic tools before surgery, the determination of the spreading, and the structural properties of the mass may designate surgical approach and prognosis.     

An Improved Operation for the Definitive Treatment of the Wolff-Parkinson-White Syndrome

As our experience increased with the definitive surgical treatment of patients with Wolff-Parkinson-White syndrome (WPW), an intraatrial method was devised that permits separation of the atria from the ventricles at any point along the annulus fibrosus. This technique depends on separating the atrium from the annulus fibrosus, using the fat about the coronary vessels and epicardial reflections from the aorta to the right atrial wall as the plane of dissection. This method allows easy approach to the septal area on the right. Successful results with 2 patients, one with Type A (left-sided) and the other with Type B (right-sided) WPW are recorded to illustrate the use of this method.     

连头婴显微分离手术中静脉窦的处理及重建

目的 报道连头婴分离手术中有关静脉窦处理及重建方法与临床效果。方法 采用显微手术分离连头婴共用的矢状窦，窦汇，横窦及直窦，用转流管分别作右婴的矢状窦，直窦与颈内静脉转流术，以及应用人造血管移植进行重建，在手术中确保左婴有完整的矢状窦，窦汇及横窦。结果 左婴术后功能恢复正常，右婴术后22h死于肾衰竭，高钾血症。结论 应用显微外科手术进行静脉窦转流和重建是连头婴分离手术成功的关键技术之一。     

Right atrial isolation: a new surgical treatment for supraventricular tachycardia. I. Surgical technique and electrophysiologic effects

This study describes the surgical technique and electrophysiologic effects of isolating the right atrium while preserving normal function and continuity of the sinoatrial node with the remainder of the heart. Thirteen adult mongrel dogs underwent normothermic cardiopulmonary bypass. A posterorlateral right atriotomy was performed that encircled the upper right atrium but excluded the atrial pacemaker complex. The incision was extended anteromedially to the tricuspid valve anulus just anterior to the membranous interatrial septum and inferiorly just posterior to the os of the coronary sinus and the tricuspid valve anulus. Postoperatively, electrophysiologic data confirmed (1) that the body of the right atrium was electrically isolated from the remainder of the heart, (2) that the sinoatrial node continued to function normally, and (3) that the sinoatrial node remained in continuity with the left atrium and ventricles. Right atrial tachycardia was simulated by rapid right atrial pacing and was confined to the isolated right atrium. Moreover, the simulated tachycardia did not affect normal sinus rhythm or normal atrioventricular conduction. It is concluded that isolation of the right atrium with preservation of normal sinoatrial node function and continuity is feasible. This technique offers an alternative to the current surgical approaches for management of refractory supraventricular tachycardias that arise in the right atrium.     

The fate of antibiotic sterilized aortic allografts in Fontan circulation: results of the long-term follow-up

Objective: Between 1977 and 1988, 27 patients, mean age 8.9 (range 4–22) received an antibiotic sterilised aortic allograft in the setting of the Fontan procedure. This study describes the long-term follow-up of these patients. Methods: Fifteen patients had tricuspid atresia, nine double inlet ventricles and three others. The connection with the allograft was made to the pulmonary artery on the right side of the aorta in ten and to a left-sided main pulmonary artery in eight. In nine patients the allograft was anastomosed between the right atrium and the right ventricle. Results: There were five early and five late deaths. One late death may have been allograft related. Survival was 81, 74 and 68% at 5, 10 and 15 years, respectively. Conduit calcification was universal. Twelve patients underwent reoperation, freedom from reoperation was 100, 88 and 54% at 5, 10 and 15 years following the initial Fontan procedure. At reoperation the gradient across the allograft was never more than 3 mmHg. The allograft was explanted with conversion to atriopulmonary or cavopulmonary connection in nine and a second allograft was inserted between the right atrium and right ventricle in two. No mortality occurred at reoperation. 41.1% of survivors still have their original allograft. Conclusions: Although there is a significant attrition rate allograft inclusion in the Fontan circulation does not change survival, but results in an increased reoperation rate. Inclusion of a valved conduit between the right atrium and ventricle does not usually enhance the growth potential of the rudimentary ventricle. All patients are in a good functional class which may represent the strict original selection criteria. The inclusion of a valve in the Fontan circulation is not recommended.     

神经导航辅助睛显微手术分离复杂连头婴畸形相嵌的脑组织

目的 报道复杂连头畸形脑组织显微分离手术方法及术中运用神经导航系统辅助的价值。方法 连头患儿颅骨多块骨窗完全形成前，左右侧患儿骨窗缘分别钉4个钛钉，再次注册，术中在导航指引下沿蛛网膜界面用显微外科技术分离脑组织，准确地寻找到直窦进行修复。结果 右患儿枕叶脑组织融合外因脑肿胀需部分切除外，2个患儿相嵌的大脑半球及小脑半球成功分离。结论 神经导航系统对复杂连头畸形深静脉窦的定位是有益的，在手术显微镜下用显微外科技术沿蛛网膜界面进行分离可减少脑组织损伤，确保手术成功。     

Simple Left Atrial Procedure for Chronic Atrial Fibrillation Associated With Mitral Valve Disease

Background. A computerized 48-channel mapping system was used to investigate the characteristics of an atrial epicardial electrogram during chronic atrial fibrillation (AF) in patients with solitary mitral valve disease. We have devised a simple left atrial procedure to eliminate the chronic AF during a mitral valve operation.

Methods. Using this mapping system, we performed intraoperative atrial mapping in 11 patients with chronic AF associated with mitral valve disease. The AF duration ranged from 0.4 to 15 years (mean, 8.0 ± 4.5 years). A simple surgical ablation of the AF on the left atrium only was performed during the mitral valve operation.

Results. The mean AF cycle length of the atria ranged from 129 to 169 milliseconds in the right atrium and from 114 to 139 milliseconds in the left atrium. The mean AF cycle length of the left atrium was shorter than that of the right atrium. Regular and repetitive activation was found in the left atria of 7 of 11 patients. The AF disappeared in all patients immediately after the operation, and 10 of these patients continued to have a sinus rhythm postoperatively (AF-free rate, 91%).

Conclusions. Computerized intraoperative mapping revealed a shorter mean AF cycle length in the left atrium. A simple left atrial procedure was effective in eliminating chronic AF associated with solitary mitral valve disease.     

Hemiarch aortic replacement for acute type A dissection in a Marfan patient with twin pregnancy

Type A aortic dissection is a high risk surgical emergency. Its occurrence during pregnancy represents an extremely high risk for death and sequelae for both the mother and the fetus. Surgical treatment in the form of cesarean delivery and aortic repair using hypothermia and circulatory arrest is mandatory in most patients. We report the case of a 29-year-old Marfan female with an acute type A aortic dissection at 34 weeks of a twin pregnancy. She underwent cesarean delivery of male twins followed by repair of the ascending aorta and proximal arch using deep hypothermia and circulatory arrest. The mother and twin babies survived without sequelae and are alive at two years.     

Right superior vena cava draining in the left atrium: anatomical, embryological, and surgical considerations

A newborn, with SDS (S = situs viscero-atrialis solitus, D = D-loop of the ventricles, S = solitus, normally related great arteries) anomalous drainage of the right superior vena cava in the left atrium, intact atrial septum, and anomalous drainage of the right superior pulmonary veins in the right superior vena cava, underwent surgical repair at our institution. This rare cyanotic, congenital, cardiac malformation is herein described with particular regard to its anatomical, embryological, and surgical implications.