Perioperative management of Fontan operation for the child with panhypopituitarism: a case report.

With the surgical improvement of congenital heart disease, Fontan operation has been applied to many complicated patients in recent years. This is the first report of a child with panhypopituitarism who underwent Fontan operation. A 5-year-old boy was scheduled for Fontan operation. He previously underwent Blalock-Taussig shunt and bidirectional Glenn operations for univentricular heart with double-outlet right ventricle and pulmonary atresia. He was receiving hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP) for panhypopituitarism secondary to removal of craniopharyngioma performed at the age of three years. Although urine output and serum sodium concentration were adequately controlled by adjustment of vasopressin infusion rate during surgery, massive pleural effusions and ascites developed postoperatively, which required several days for control by adjusting the dose of oral DDAVP and normalize the serum sodium level. Intraoperative management of Fontan operation for a patient with panhypopituitarism was controllable by appropriate hormone replacement. However, postoperative fluid management was complicated by the clinical features of panhypopituitarism and Fontan physiology.

Influence of the aldehyde dehydrogenase 2 polymorphism on the vasodilatory effect of nitroglycerin in infants with congenital heart disease and pulmonary arterial hypertension.

PURPOSE: The influence of the aldehyde dehydrogenase 2 (ALDH2) gene polymorphism on the pharmacokinetics and haemodynamics of nitroglycerin (GTN) was determined in human subjects. METHODS: Eighteen infants (nine each with and without ALDH2 gene polymorphism) with congenital heart disease and pulmonary arterial hypertension participated in this study. GTN treatment started at a dose of 2 µg/kg/min, and the dose was escalated by 1-2 µg/kg/min until pulmonary vascular resistance (PVR) was reduced by more than 30%. The plasma GTN concentration and PVR were measured at the end of each infusion period. RESULTS: Plasma GTN concentrations were significantly higher in patients with the ALDH2 gene polymorphism than in those without the polymorphism. Conversely, the reduction in PVR was smaller in patients with the ALDH2 gene polymorphism than in those without. CONCLUSIONS: These data suggest that the ALDH2 gene polymorphism influences the pharmacokinetics and haemodynamics of GTN in human subjects.

[Idiopathic Pulmonary Artery Aneurysm].

Pulmonary artery aneurysm (PAA) is usually associated with congenital heart disease, pulmonary artery hypertension, and connective tissue abnormalities, but idiopathic PAA is a rare clinical entity. We experienced a surgical case of idiopathic PAA measuring 60 mm in diameter. A 72-year-old man had been admitted to a nearby hospital 5 years before because of an abnormal shadow on chest X-ray, and was diagnosed with an idiopathic PAA measuring 37 mm in diameter. The PAA gradually expanded during follow-up and surgery was scheduled. Aneurysmal resection and reconstruction with a 24 mm expanded polytetrafluoroethylene graft were performed. The postoperative course was uneventful and the patient was discharged on the 11th postoperative day.

[Serial Pathological Changes Between Pre- and Post-pulmonary Artery Banding in a 21-trisomy Patient with Ventricular Septal Defect].

Although many patients with a ventricular septa defect undergo primary intra-cardiac repair surgery, some patients require 2-stage repair with pulmonary artery banding. Patients with 21-trisomy, who exhibit fragility of the pulmonary vasculature and respiratory systems, may be candidates for this approach. Herein, we report the case of a 21-trisomy patient with ventricular septal defect associated with pulmonary hypertension. She underwent pulmonary artery banding at 4 months of age, and a subsequent radical operation at 27 months. Serial pathological findings between pre- and post-pulmonary artery banding revealed that changes of pulmonary vasculature and respiratory systems were reversible. Her Heath-Edwards grade changed from 0 from II and index of pulmonary vasculature disease changed from 1.1 to 1.0. In conclusion, pulmonary artery banding in a case with pulmonary hypertension prevented the lung from becoming irreversible pathological changes.

[Tetralogy of Fallow with Total Anomalous Pulmonary Vein Return and Atrial Septal Defect;Successful Two-staged Surgical Management;Report of a Case].

A combination of tetralogy of Fallot( TOF) and total anomalous pulmonary venous return(TAPVR) is rare and results in chronic volume and pressure load of the right side of the heart and underfilling of the left heart. We report a successful 2-staged surgical correction of TOF associated with TAPVR and atrial septal defect. The patient was unsuitable for total primary intracardiac correction because the volume of the left ventricle was considered to be small. First, repair of anomalous pulmonary venous return and palliative right ventricle outflow tract reconstruction were simultaneously performed in 2 months of birth. One year after 1st operation, cardiac catheterization revealed that normalization of left ventricle volume, so 2nd operation was planned. Total correction of ventricular septal defect and right ventricle outflow reconstruction was performed and the patient was discharged on the 21st postoperative day with good hemodynamic status.

[Maze procedure in a case of dextrocardia with atrial septal defect and persistent left superior vena cava].

A 52-year-old man was diagnosed with dextrocardia at the age of 1 year and was asymptomatic until 1 year before admission. He was transferred to our hospital for management of atrial fibrillation. A transthoracic echocardiogram showed dextrocardia with atrial septal defect;moderate tricuspid valve regurgitation; and a large, persistent left superior vena cava. A cardiac catheterization study revealed that pulmonary flow/systemic flow (Qp/Qs) was 3.6 and that pulmonary vascular resistance was 2.5 Wood U·m². Intracardiac repair with tricuspid annuloplasty and a maze procedure was scheduled. When establishing cardiopulmonary bypass, venous drainage was initially obtained from the inferior vena cava and the left superior vena cava, and the small superior vena cava was then directly cannulated after opening the right atrium. The patient's postoperative course was uneventful, and serial electrocardiograms have demonstrated maintenance of normal sinus rhythm for 3.5 years after the operation.

Survey of reoperation indications in tetralogy of fallot in Japan.

BACKGROUND: Although the survival rate for repaired Tetralogy of Fallot (TOF) is dramatically improving, anatomical and functional residua and sequelae followed by arrhythmias and sudden death are still challenging issues to be resolved. Reoperation can reduce the incidence of arrhythmias and sudden death, but there is no consensus on the indications of reoperation for patients with TOF, especially in Japan. METHODS AND RESULTS: A cross-sectional questionnaire survey of reoperation indications in patients with TOF was performed through a Japanese multicenter study. The questionnaire, which focused on the number of repaired TOF patients aged >15 years old, reoperation indications and management, was sent to the institutions belonging to Japanese Society for Adult Congenital Heart Disease. In total, 41.5% (78/188) of the institutions replied. The total number of repaired TOF patients was 4,010, and sudden cardiac death was observed in 45.236/4,010 (5.9%) experienced reoperation. Pulmonary stenosis (32%) and pulmonary regurgitation (29%) were the most common reasons for reoperation. There were only 2 implantable cardioverter defibrillator or resynchronization therapy defibrillator implantations. The physiological/anatomical indications of reoperation differed among the hospitals. CONCLUSIONS: Approximately 1.1% of patients suffered sudden death and 6% of repaired TOF patients had reoperation. The indications of reoperation, however, varied among the institutions. Therefore, detailed information for each case of sudden death or reoperation should be collected and analyzed in order to establish guidelines for reoperation.

[Cardiac papillary fibroelastoma on the Chiari's network of a 3-month-old baby; report of a case].

A 3-month-old boy was transferred to our hospital because of a cardiac tumor in the right heart, which had been prenatally diagnosed by fetal echocardiography. During the operation, the tumor was found on the Chiari's network in the right atrium. The tumor was excised and the ventricular septal defect was repaired. Postoperative histological examination revealed that the tumor was a papillary fibroelastoma. He was transferred to a pediatric unit for postoperative care.

Successful Management in an Infant Patient of PHACE Syndrome with a Complicated Aortic Arch Anomaly.

PHACE syndrome is a congenital disorder often associated with a cervicofacial infantile hemangioma and complicated cardiovascular malformations. Patients with PHACE syndrome often have complex aortic arch anomalies, longer aortic stenosis or agenesis segments, and increased vascular tortuosity; therefore, perioperative management and surgical repair are challenging. We report a case of a female infant with PHACE syndrome and complex cardiovascular anomalies such as a double aortic arch associated with interruption of the left aortic arch, coarctation of the right aortic arch, patent ductus arteriosus, ventricular septal defect, and atrial septal defect. She was born at 36 weeks of gestation (birth weight, 2,150 g) and the diagnosis was confirmed by three-dimensional computed tomography. Because her patent ductus arteriosus did not close at first, her heart failure was managed preoperatively without prostaglandin E 1. We initially attempted to promote weight gain. Surgical planning and simulation were performed using the patient-specific three-dimensional cardiovascular model created from computed tomography data. She underwent a successful aortic arch reconstruction by an end-to-side anastomosis with anterior patch augmentation at the age of 56 days. Detailed planning and simulation before surgery were vital in achieving favorable outcomes. Careful management and surgical planning using a patient-specific three-dimensional model are vital, especially in patients with complex malformations, such as in our case.

Cardiac Arrest in a 33-year-old Marathon Runner with Anomalous Right Coronary Artery Originating from the Pulmonary Artery.

A 33-year-old marathon runner presented with anomalous right coronary artery originating from the pulmonary artery after being admitted for cardiac arrest. Surgical re-implantation of the right coronary artery to the aortic root to re-establish right coronary ostial circulation was successful. The patient resumed exercise and required no further medical therapy.

[Cyanotic congenital heart disease. Part 1].

This section deals with subgroups of cyanotic complex congenital heart diseases, most present early in life during neonatal period or early infancy. And some present with ductal dependency for survival. Here included are the complete transposition of the great arteries (TGA) [or Taussig-Bing anomaly] with/without aortic arch obstruction, total anomalous pulmonary venous connection (TAPVC), pure pulmonary atresia/stenosis (PPA/PPS) [pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis], hypoplastic left heart syndrome (HLHS) and its variants. In d-TGA cases except those with left ventricular outflow obstruction, primary Jatene procedure with/without concomitant repair of the aortic arch obstruction is the procedure of choice with good surgical and long-term results although acceptable rate of re-intervention is mandatory. In TAPVC, primary repair usually goes with good long-term result, although minority of the patients eventually develops for midable complication, postoperative pulmonary venous obstruction. Sutureless pericardial repair has been developed for coping with and later for prevention of this complication with promising mid-term results. Its introduction in primary repair especially in complex cases appears around the corner. HLHS and its variants has been the most challenging group of patients requiring staged approach starting with Norwood procedure or bilateral pulmonary artery banding. Right ventricle-to-pulmonary artery conduit, introduced as a Japanese innovation, has contributed marked improvement of surgical results although further refinements surely are necessary. Patients with PPA/PPS have a wide range of clinical pictures with intrinsic problems of right ventricle, tricuspid valve and associated coronary artery abnormalities. Adequate use of single ventricle physiology in many cases offers fair surgical results.

Long-term survival without surgical intervention in a patient with a natural history of a single right ventricle: A case report.

Single ventricle (SV), a complex cardiac anomaly, if left untreated, is thought to lead to a poor prognosis. Herein, we report the case of a long-term survivor with an unrepaired SV with right ventricle (RV) morphology. A 55-year-old man presented with a SV with RV morphology, with a double outlet, large atrial septal defect, common atrioventricular valve, pulmonary valve stenosis (PS), and dextrocardia. Because the native PS provided adequate restriction of the pulmonary blood flow, he did not develop pulmonary hypertension; however, he had severe cyanosis. In patients with SV and moderate PS, even if the SV has RV morphology, long-term survival may be possible without surgical intervention. .

Age-dependent vulnerability to ischemia-reperfusion injury of cyanotic myocardium in a chronic hypoxic rat model.

This study evaluated the effects of chronic hypoxia from birth on the resistance of rat hearts to global ischemia, with special emphasis on the duration of hypoxia. Male Wistar rats were housed from birth for 4 weeks or 8 weeks either in a hypoxic environment (FiO2 = 0.12) or in ambient air (8 animals for each group). Isolated rat hearts were perfused for 40 min with oxygenated Krebs-Henseleit buffer, subjected to 20 min global no-flow ischemia at 37, and then underwent 40 min of reperfusion. A non-elastic balloon was inserted into the left ventricle and inflated until the pre-ischemic LVEDP rose to 8 mmHg. Cardiac function was measured before and after ischemia. The post-ischemic percent recovery of LVDP in hypoxic hearts was worse than in normoxic hearts (4 weeks:55+/-7 vs. 96+/-3%, p0.01;8 weeks:40+/-5 vs. 92+/-4%, p0.01), and was worst in the 8-week-hypoxic hearts. Similarly, the percent recovery of dP/dt in the hypoxic hearts was lower than in the normoxic hearts (4 weeks:51+/-5 vs. 96+/-7%, p0.01;8 weeks:31+/-6 vs. 92+/-7%, p0.01), and was lowest in the 8-week-hypoxic hearts. In conclusion, cyanotic myocardium revealed an age-dependent vulnerability to ischemia-reperfusion injury in a chronic hypoxic rat model.

Repair of a dysplastic tricuspid valve using artificial chordae: case report.

Atrioventricular valve repair with artificial chordal replacement has been widely used for congenital and acquired mitral valve abnormalities, but not for tricuspid valve abnormalities. A case is presented of dysplastic tricuspid valve that was successfully repaired using artificial chordae. A 2-year-old female presented with poor weight gain. Echocardiography revealed severe tricuspid regurgitation due to dysplastic tricuspid valve, poor coaptation by prolapse of the anterior leaflet, and tethering of the septal leaflet by short chordae. The prolapsed anterior leaflet was repaired with three pairs of 6-0 expanded polytetrafluoroethylene sutures. The short chordae of the septal leaflet were detached, and the septal and posterior leaflets were sutured together. Trivial tricuspid regurgitation was noted postoperatively. There was no tricuspid regurgitation during the follow up period of three years. The present case provides further evidence that artificial chordal replacement is a useful technique even for small children with congenitally abnormal tricuspid valves.

Continuous cerebral and myocardial perfusion during aortic arch repair in neonates and infants.

To minimize myocardial ischemia, we repaired aortic arch obstruction with ventricular septal defect, using two different techniques of cerebral and myocardial perfusion. Seventy-one infants, ages 3 to 137 days, underwent primary repair of coarctation of the aorta (n = 49)/interruption of the aortic arch (n = 22) with ventricular septal defect. In 65 patients, an end-to-end arch anastomosis was performed with cerebral and myocardial perfusion through the innominate or the ascending arterial cannula (non-working beating heart: NWBH). In the remaining 6 patients, an arterial cannula was placed into the innominate artery. With partial cardiopulmonary bypass, the innominate artery was snared proximal to the cannulation site and the ascending aorta was cross-clamped. An extended arch anastomosis was carried out with cerebral perfusion and a working beating heart (WBH). Ten patients (15%) undergoing aortic arch repair with the NWBH technique required cardioplegic arrest to complete a proximal anastomosis, whereas in all 6 repairs with the WBH technique, the extended anastomoses were completed without myocardial ischemia. One hospital death and late death occurred, with an overall survival of 98%. End-to-end arch reconstruction is feasible without myocardial ischemia, using the NWBH technique in patients without hypoplastic arch and using the WBH technique in patients with hypoplastic arch.

Advantages of temporary venoatrial shunt using centrifugal pump during bidirectional cavopulmonary shunt.

Single-ventricle palliation without the use of cardiopulmonary bypass carries advantages that reduce systemic edema and inflammatory responses; however, simple clamping of the superior vena cava (SVC) without a temporary shunt leads to increase in cerebral venous pressure and subsequent decrease in cerebral blood flow during bidirectional cavopulmonary shunt (BCPS). We report our experience of BCPS, using a centrifugal pump-assisted temporary shunt. The criteria included an unrestrictive interatrial communication, the absence of atrioventricular valve regurgitation, and the existence of an antegrade pulmonary blood flow. From August 2000, 14 children with single-ventricle physiology met the criteria. The mean age was 1.0 +/- 0.9 years, and the mean weight was 8.4 +/- 2.6 kg. A temporary shunt was established between the SVC and the right atrium with right-angle cannulae, which were connected to a centrifugal pump to accelerate the blood flow from the SVC to the right atrium. All patients tolerated the procedure. Mean central venous pressure was 17 +/- 4 mm Hg, and transcutaneous oxygen saturation was maintained at 77 +/- 8% during anastomosis. No patients required blood transfusion. There were no postoperative neurological complications. The centrifugal pump-assisted temporary shunt offered safer and more effective circulatory support than other shunt systems, with excellent venous drainage in pediatric patients undergoing BCPS.

Protein-losing enteropathy seven years after total right ventricular exclusion procedure for arrhythmogenic right ventricular dysplasia.

A 27-year-old man who was diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) underwent the total right ventricular (RV) exclusion procedure: the RV free wall was resected and an extracardiac total cavopulmonary connection (TCPC) was created using a 24-mm-diameter polytetrafluoroethylene (PTFE) graft. After an uneventful period of 7 years, he began to develop protein-losing enteropathy (PLE), which was resistant to medical therapy, moderate mitral regurgitation (MR) and right femoral arteriovenous fistula due to heart catheterization. Therefore, fenestration of TCPC, mitral annuloplasty and ligation of arteriovenous fistula were performed. After the operation, symptoms and levels of total protein and albumin were immediately improved and normalized.

Total right ventricular exclusion improves left ventricular function in patients with end-stage congestive right ventricular failure.

BACKGROUND: We developed a total right ventricular (RV) exclusion procedure for the treatment of isolated congestive RV failure. The objective of the present study was to elucidate the effects of a complete removal of RV volume overload (RVVO) on the surgically created single left ventricle (LV). METHODS AND RESULTS: Three adults (2 arrhythmogenic RV dysplasia, 1 Ebstein) and 5 children (all Ebstein) in NYHA class IV underwent the procedure. The RV free wall was resected from the heart, and the tricuspid orifice was closed. Pulmonary blood supply was obtained by a cavopulmonary connection in 6 patients and a systemic-pulmonary shunt in 2. The LV function was evaluated by 2-dimensional echocardiography 1 month after the surgery. All patients are alive. The paradoxical movement of the interventricular septum and geometry of the LV expressed by its eccentricity (2.1 to 1.2, P<0.01) were normalized after the operation in all 8 patients. LV end-diastolic volumes (59% to 109% of normal value, P<0.01), indexed maximal left atrial area (6.5 to 10.5 cm2/m2, P<0.01), LV ejection fraction (27% to 62%, P<0.01), and cardiac index (2.1 to 3.3 L/min/m2, P<0.05) all significantly increased. CONCLUSIONS: Removal of the RVVO by means of the total RV exclusion procedure provides effective volume loading, restores a cylindrical shape, and improves contractile function of the LV, thus leading to increased systemic output.

Usefulness of helical computed tomography in diagnosing pulmonary vein stenosis in infants.

We investigated the usefulness of helical computed tomography(CT)in the morphological diagnosis of pulmonary vein stenosis, particularly that in infants and small children. In total, 20 helical CT examinations were performed in 10 post-operative cases of Total Anomalous Pulmonary Venous Drainage(TAPVD), 3 cases of single right ventricle, and 1 case of single left ventricle. In all cases, distinct morphological imaging was possible. Pulmonary vein stenosis could be categorized into three types: (1)stenosis from the anastomosis of the common pulmonary vein (CPV)-the left atrium (LA) to the peripheral pulmonary vein; (2) stenosis only at the anastomosis of CPV-LA; and (3) stenosis due to compression by nearby organs. Coronal views by multiplanar reconstruction (MPR) provided morphological information along the up-down direction of the body axis. Morphological diagnosis of pulmonary vein stenosis is important in deciding prognosis and therapeutic regimens, and helical CT was considered useful for such diagnosis in our 14 young patients.