Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Unroofed coronary sinus syndrome

Unroofed coronary sinus syndrome or coronary sinus septal defect is a rare congenital cardiac anomaly. We performed corrective operations in 9 patients with such condition. Of them, seven patients had the completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) directly draining into the left atrium (LA). One patient had PLSVC and the partially unroofed terminal portion of the coronary sinus (CS), or sinus ostium open into LA. Another patient had total anomalous pulmonary vein connection and the partially unroofed mid-portion of CS without PLSVC. The operative methods of the unroofed coronary sinus included: ligating PLSVC and repairing ostium primum or secundum atrial septal defects in 4 cases; creating an intra-atrial tunnel from PLSVC to the right atrium (RA) and repairing the atrial septal defect in 2 cases; reconstructing the intra-atrial septal with a patch as a baffle to guide PLSVC or sinus ostium respectively towards RA in 2 cases; enlarging the defect on the coronary sinus roof and repairing the atrial septal defect of the coronary sinus type in one case. No patient died in this group. Because unroofed coronary sinus syndrome has atypical clinical manifestation and usually complicates varied congenital anomalies, its preoperative diagnosis is very difficult in some times and may draw the reliable support from echocardiography and catheterization. The operative program and method must depend not only upon its type but also upon PLSVC presence of absence, and communication between PLSVC and RSVC.     

Two cases of scimitar variant

The scimitar sign is characteristic of partial anomalous pulmonary venous drainage into the inferior vena cava (IVC). We encountered two variant cases of scimitar sign. In one case, the scimitar vein entered both the IVC and the left atrium (LA) without any intracardiac shunts. Surgical repair was made by simple ligation of the scimitar vein to correct the left to right shunt. Retrograde balloon occlusion angiography of the scimitar vein was diagnostic. In the other case, the scimitar vein showed a meandering course, and then drained into the LA without any connection with the IVC, and surgical intervention was not required.     

Diversion of the inferior vena cava into the left atrium after repair of atrial septal defect with partial anomalous pulmonary venous drainage

An 11-year-old female had operative repair of atrial septal defect associated with partial anomalous pulmonary venous drainage by direct suture at 6-year-old. Postoperatively, cyanosis and nodal bradycardia were noted by physical exercise. During 5 years thereafter, the symptoms were gradually aggravated accompanying. Cardiac catheterization revealed diversion of the inferior vena cava into the left atrium. Oxygen sampling showed right to left shunt of 22% at the atrial level. At reoperation, orifice of the inferior vena cava into the right atrium became narrow 5 mm in diameter. After enlargement of the orifice, the atrial septum was created with a Gore-Tex patch to redirect the inferior vena cava to the right atrium. Post operative course was uneventful with disappearance of the cyanosis. The patient is up and well now 9 years after reoperation.     

Management of incomplete spontaneous abortion with suction curettage in the pediatric emergency department

Medical records, angiograms and operative records of 28 patients with partial anomalous pulmonary venous connection (PAPVC) were reviewed. Twenty patients had one anomalous pulmonary vein (APV), and 8 had more than two APVs. Twenty-five patients (89%) had APVs originating from the right lung, 2 (7%) from the left lung and 1 (4%) from both lungs. In the 25 patients with APVs originating from the right lung, 9 had APVs draining into the superior vena cava (SVC), 13 into the right atrium (RA), 1 into the inferior vena cava (IVC) and 2 into both the SVC and RA. In the 2 patients with APVs originating from the left lung, 1 had APVs draining into the RA, and the other had APVs draining into the innominate vein. The patient with APVs originating from both lungs had connection to the IVC. Twenty-three patients (82%) had additional cardiovascular defects. Surgery was performed in 13 patients who had pulmonary/systemic flow ratios greater than 2.0. The patients have done well after surgery. In 7 patients, we were unable to accurately determine the number or sites of drainage of APVs prior to surgery. We conclude that selective pulmonary angiography is indispensable for the accurate diagnosis of PAPVC.     

Handedness assessment inventory

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Cryoablation: a novel approach to neurolysis of the ganglion impar

BACKGROUND: The main goal in the surgical repair of total anomalous pulmonary venous drainage is to reestablish a wide patent connection between the common pulmonary vein and the left atrium. Several techniques have been proposed for achieving this objective, each of which has advantages and disadvantages. The superior approach between the superior vena cava and the ascending aorta was introduced in 1976 for the repair of supracardiac forms of total anomalous pulmonary venous drainage, but it often provides a less than optimum exposure, particularly in tiny infants. We proposed a modification of this approach that includes division of the ascending aorta and offers excellent exposure. METHODS: Seventeen patients (15 neonates and 2 infants) with supracardiac total anomalous pulmonary venous drainage (n = 13) or mixed forms of total anomalous pulmonary venous drainage (n = 4) underwent surgical repair with the use of the modified superior approach. Circulatory arrest was not required in 10 patients and the mean cross-clamp time was 32.5 +/- 13.8 minutes. RESULTS: There was 1 postoperative death resulting from intractable pulmonary hypertension in a compromised infant who was referred to our unit receiving extracorporeal membrane oxygenation. One patient with common hypoplasia underwent reoperation twice at 2 months and then 3 months after the first procedure. All the other patients had a smooth postoperative course, and midterm evaluation showed a widely patent anastomosis between the common vein and the left atrium. CONCLUSIONS: The modified superior approach for the repair of supracardiac total anomalous pulmonary venous drainage can be useful to enhance exposure during surgical repair and may contribute to improved patient outcome.     

Technique of transplantation for hypoplastic left heart syndrome with left superior vena cava

The presence of a left superior vena cava in infants with hypoplastic left heart syndrome complicates the technical performance of orthotopic cardiac transplantation. In this report we describe a modification of the recipient cardiectomy to maintain patency of the left superior vena cava, leaving the recipient coronary sinus as a conduit for it into the right atrium and avoiding complex venous reconstructions.     

Early coronary sinus reroofing using the left atrial baffle

Surgical correction of unroofed coronary sinus, left superior vena cava, dextrocardia, and situs solitus in a 4-month-old infant consisted of reroofing the coronary sinus by means of a left atrial flap while redirecting the left superior vena cava to the right atrium. Excellent access to the left side of the left atrium was afforded by the associated dextrocardia.     

Pulsatile total cavopulmonary shunt for hypoplastic right heart syndrome with abnormal systemic venous return--a case report

A pulsatile total cavopulmonary shunt was successfully performed on a 5-year-old girl with hypoplastic right heart syndrome associated with abnormal systemic venous return; at the same time, modified mitral valve replacement was performed for mitral regurgitation. The right atrium, tricuspid valve and right ventricle were all extremely dimunitive. The diameter of the tricuspid valve was 50% of normal and the volume of the right ventricle was 8.6% of normal. In addition, there were severe subpumonary stenosis, a restrictive ventricular septal defect (VSD) and an atrial septal defect (ASD). The bilateral superior venae cavae (SVCs) and the hepatic vein drained to the left atrium, and the inferior vena cava was infrahepatically interrupted with a hemiazygos connection to the left superior vena cava. At the operation, each SVC was anastomosed end-to-side to each branch of the pulmonary artery (PA). The restrictive ventricular septal defect and stenotic subpulmonary lesion were left. The diameter of the ASD was reduced from 12 mm to 7 mm. The main PA was neither divided nor banded. The pulsatile blood flow from the left heart to the PA was regurated by a native restrictive VSD and stenotic subpulmonary lesion, and that from the right heart via the ASD was limited by reducing the size of the ASD. These described anatomic arrangements produced adequate antegrade pulsatile flow in the PA, which might prevent the development of pulmonary arteriovenous fistulae and, besides permit transfer of drainage of the hepatic vein from the left to the right atrium via the ASD in future.     

Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage

The correction of shunts resulting from partial anomalous pulmonary venous drainage has become an accepted surgical procedure. Surgical complications, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual complications resulting from this type of surgery. Unilateral pulmonary venous obstruction and repeated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an indaequate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.     

Uptake of pre-emergent herbicides by corn: distribution in plants and soil

We experienced an uncommon case of coronary sinus atrial septal defect, which was not associated with a left superior vena cava (LSVC). A 29-year-old woman, who experienced fatigue during moderate exercises, was found to have the atrial septal defect located in the lower part of the septum by echocardiography preoperatively. On operation, the coronary sinus ASD was diagnosed. The ostium of coronary sinus was located in the left atrium. This type is classified as partially unroofed coronary sinus without LSVC. Our case, in the absence of the LSVC, was repaired by simple closure of the coronary sinus ASD, so the coronary veins drained into the left atrium. Postoperative course was uneventful.     

Atrial septal displacement for repair of anomalous pulmonary venous return into the right atrium

BACKGROUND: In the repair of anomalous connection of the pulmonary veins to the right atrium, the use of a baffle of pericardium to divert the pulmonary venous blood into the left atrium could cause pulmonary venous obstruction as a result of thickening of the pericardial patch. Anomalous pulmonary venous drainage to the right atrium caused by malposition of the atrial septum primum can be repaired by displacing the shifted septum primum to the normal position. METHODS: In 5 patients with total (n=2) or partial (n=3) anomalous pulmonary venous drainage into the right atrium, the septum primum was shifted toward the left atrium and the pulmonary veins drained into the anatomic right atrium despite their normal connection with the posterior wall of the left atrium. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum between the anomalous pulmonary veins and both venae cavae. No patch was used. RESULTS: Postoperative echocardiography showed a wide pathway from the pulmonary veins to the left atrium with no stenotic portions. No atrial arrhythmias occurred after the operation. CONCLUSIONS: This technique may be advantageous because it allows for future growth of the route of the pulmonary venous pathway and avoids postoperative supraventricular arrhythmias.     

Mitochondrial gene mutations and human diseases: a prolegomenon

OBJECTIVE: To compare "central venous pressure" in pediatric patients in a clinical setting as measured from catheters in the infrahepatic inferior vena cava and the right atrium. DESIGN: Prospective, unblinded study. SETTING: Cardiothoracic intensive care unit of a tertiary care university hospital. PATIENTS: Thirty-three pediatric cardiac surgical patients, 2 days to 92 months of age (mean 24 +/- 4 months). INTERVENTIONS: All patients had intraoperative placement of an 8-cm, double-lumen, femoral venous catheter and a transthoracic right atrial catheter. Patients were studied for 0 to 2 days after surgery. MEASUREMENTS AND MAIN RESULTS: Measurements were obtained during mechanical and spontaneous ventilation. Although not statistically identical, measurements of "central" venous pressure in the inferior vena cava and right atrium correlated well (r2 = .87 for mechanical ventilation; r2 = .83 for spontaneous ventilation). Of 31 data pairs in mechanically ventilated patients, the absolute difference in pressures was as large as 3 mm Hg in three patients and <3 mm Hg in all the rest. In 15 spontaneously breathing patients, there were only three data measurements where the difference in pressure was 2 mm Hg and none of the differences was greater. In spontaneously breathing patients, the phasic changes due to respiratory variations in venous pressure were in phase in both the intrathoracic and intra-abdominal catheter positions. CONCLUSIONS: We conclude that while "central" venous pressures measured in the inferior vena cava and in the right atrium are not statistically identical, any differences are well within clinically important limits. Placement of central venous pressure catheters in the inferior vena cava by the femoral venous approach is a reliable alternative to cannulating the superior vena cava in pediatric patients without clinically important intra-abdominal pathology and with anatomic continuity of the inferior vena cava with the right atrium. Relatively short femoral vein catheters allow adequate measurement of central venous pressure without concern for exact catheter tip position and without the risk of right atrial perforation, intracardiac arrhythmias, and inadvertent puncture of carotid and intrathoracic structures. Unlike previously reported results in neonates, we found that the phasic changes of venous pressure with the respiratory cycle were similar in both intrathoracic and intra-abdominal recordings, making this an inappropriate clinical indicator of venous catheter tip position.     

Successful ablation of atrioventricular nodal reentry tachycardia in a patient with persistent left superior vena cava

We report the case of a patient with atrioventricular nodal reentry tachycardia associated with persistent left superior vena cava draining to a large coronary sinus. Successful ablation was performed at the level of the superior lip of the coronary sinus ostium.     

VDD pacing in persistent left superior vena cava

Although persistent left superior vena cava (PLSVC) is the most common major venous anomaly of the heart, associated absence of the right superior vena cava with normal visceral situs is exceedingly rare. Such a patient presented with complete heart block requiring permanent pacing. This was achieved successful using a single lead VDD system via the PLSVC with atrial sensing in the coronary sinus.     

Nitric oxide and the intestinal epithelial barrier: does it protect or damage the gut?

The right and left venous valves of the sinoatrial orifice regulate the flow of blood from the sinus venosus to the atrium in pisces, amphibians, and reptiles. In aves, mammals and humans, the venous valve loses its hemodynamic function and hence has become the 'Cinderella' among heart valves, receiving attention only when congenital anomalies occur that are related to it. The right venous valve persists in humans as the crista terminalis, Eustachian and Thebesian valves. An intraluminal muscle band is described located inside the right atrium, and coursing in the line of the crista terminalis between the septum spurium and inferior vena cava. This abnormal band is possibly derived from the right venous valve.     

Surgical treatment of the massively dilated ureter in children. Part II. Management by primary reconstruction

Bypass of the left ventricle was accomplished in dogs and the entire circulation was supported temporarily by only the right ventricle. The atrial septum was excised, and the atrium was repartitioned so that the pulmonary veins were in continuity with the right ventricle and the venae cavae were connected through the atrium. Anastomosis of the superior vena cava to the right pulmonary artery brought systemic venous return directly to the lungs. The main pulmonary artery was ligated proximal to the bifurcation, preserving distal confluence of right and left pulmonary arteries. A tubular prosthesis between the proximal pulmonary artery and the aorta connected the right ventricle to the systemic circuit. This procedure, or some modification of the principle, may have clinical feasibility in the treatment of patients with hypoplastic left heart syndrome.     

Extracardiac direct total cavopulmonary connection

We report a successful extracardiac direct total cavopulmonary connection without prosthetic materials performed in a 3-year-old boy with tricuspid atresia, infundibular pulmonary stenosis, and normally positioned great arteries. The transected pulmonary trunk was bought down posterolaterally with respect to the right atrium and was anastomosed end-to-end with the inferior vena cava.     

Grouper--creation of marker sets for multiplexed genotyping

We report the successful insertion and subsequent retrieval of a Gunther-Tulip vena cava filter in a patient with an anomalous left-sided inferior vena cava, who developed a right ilio-femoral venous thrombosis prior to planned surgical resection of a right femoral osteosarcoma. The indication was for short-term prophylaxis against pulmonary embolism during manipulative leg surgery.