Hideki Uemura

Total Anomalous Pulmonary Venous Connection: Morphology and Outcome From an International Population-Based Study

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. # Clinical Perspective {#article-title-32}

Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both

Abstract We conclude, therefore, that description and analysis of visceral heterotaxy need to include considerations of the bodily organs as well as attention to cardiac structure. It must then be recognized that important differences exist between the groupings made on the basis of splenic status and morphology of the appendages. It is up to the individual investigator, based on the specific topic to be studied, to choose between the utilities of these different approaches.

Double switch operation in cardiac anomalies with atrioventricular and ventriculoarterial discordance

Since June 1987, 10 of 19 consecutive patients with atrioventricular and ventriculoarterial discordance (average age 4 +/- 2 years) had undergone a double switch operation with the morphologically left ventricle used as a systemic ventricle. There were two combinations of procedures. Atrial switch combined with arterial switch was used in two patients who had a normal pulmonary valve. Atrial switch combined with ventriculoarterial switch by Rastellis procedure was used in eight patients with pulmonary stenosis or atresia and a large ventricular septal defect. One early death and two late deaths have occurred in a postoperative follow-up period of up to 4 years. Subsequent problems were mainly related to the results of atrial switch procedures in patients who had a small atrium because of low pulmonary flow, especially in patients with apicocaval juxtaposition. Our experience suggested that the double switch operation would open a new era of definitive surgical treatment in half of the patients with atrioventricular and ventriculoarterial discordance.

What factors affect ventricular performance after a Fontan-type operation

Postoperative conditions after a Fontan-type operation, particularly as they affect results in the early term, are thought to depend on factors such as the state of pulmonary circulation and ventricular function. In this study, we attempted to determine the factors that influence ventricular characteristics in the middle term after Fontan-type procedures. Catheterization was performed at a mean of 15 months after operation in 57 patients with univentricular atrioventricular connection who underwent the operation between 1.0 and 22.6 years of age. End-diastolic volume, end-systolic volume, ejection fraction, and end-diastolic pressure of the systemic ventricle were analyzed together with an estimation of the systemic flow index. These parameters were influenced significantly by the presence of atrioventricular valve insufficiency. The morphologically left ventricle showed a better ejection fraction than did the morphologically right ventricle, whereas the systemic flow index was greater in patients undergoing total cavopulmonary connection than in those receiving an atriopulmonary connection. Young age was significantly associated with a better postoperative contractility, whereas the potential for impaired ventricular compliance was suggested in several patients undergoing operation after 4 years of age. On the basis of our results, we conclude that total cavopulmonary connection performed at a young age should be the surgical procedure of choice and that atrioventricular insufficiency must be treated properly at, and even after, the initial definitive repair.

Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation

OBJECTIVES This study investigated the influences of ventricular morphology, hemodynamics and clinical findings on exercise capacity in patients after the Fontan operation. BACKGROUND Determinants of exercise capacity after the Fontan operation remain unclear. METHODS Peak oxygen uptake (PVo2) was determined in 105 patients by exercise test and compared to hemodynamics and clinical findings. Patients were divided into three groups based on ventricular morphology: those with a right ventricle (group RV), a biventricle (group BV) and a left ventricle (group LV). RESULTS Ten patients with atrioventricular valve regurgitation (AVVR) or hypoxia exhibited a low PVo2. After excluding these patients, although PVo2 did not correlate with hemodynamics, except ventricular ejection fraction (p < 0.02), it correlated with age at the Fontan operation and exercise test (p < 0.002). The PVo2 was higher in group LV (63+/-9%) than in groups RV (55+/-9%) and BV (55+/-12%) (p < 0.01), while an inverse correlation between PVo2 and age at operation was demonstrated only in group RV (p < 0.05). Groups RV or BV and age at exercise test were associated with a lower PVo2, whereas group LV was an independent predictor of a higher PVo2 (p < 0.01). During 4.2 years of follow-up, a decrease in peak heart rate was related to a decrease in PVo2 (p < 0.05). The PVo2 decreased in group RV (p < 0.01). CONCLUSIONS In addition to AVVR, hypoxia, and heart rate response, ventricular morphology is related to exercise capacity. Early Fontan operation may be beneficial in terms of exercise capacity, especially in the group RV patients.

Clinical Outcomes of Surgical Pulmonary Valve Replacement after Repair of Tetralogy of Fallot and Potential Prognostic Value of Preoperative Cardiopulmonary Exercise Testing

Background— Indications for surgical pulmonary valve replacement (PVR) after repair of tetralogy of Fallot have recently been broadened to include asymptomatic patients. Methods and Results— The outcomes of PVR in adults after repair of tetralogy of Fallot at a single tertiary center were retrospectively studied. Preoperative cardiopulmonary exercise testing was included. Mortality was the primary outcome measure. In total, 221 PVRs were performed in 220 patients (130 male patients; median age, 32 years; range, 16–64 years). Homografts were used in 117 patients, xenografts in 103 patients, and a mechanical valve in 1 patient. Early (30-day) mortality was 2%. Overall survival was 97% at 1 year, 96% at 3 years, and 92% at 10 years. Survival after PVR in the later era (2005–2010; n=156) was significantly better compared with survival in the earlier era (1993–2004; n=65; 99% versus 94% at 1 year and 98% versus 92% at 3 years, respectively; P=0.019). Earlier era patients were more symptomatic preoperatively (P=0.036) with a lower preoperative peak oxygen consumption (peak O2; P<0.001). Freedom from redo surgical or transcatheter PVR was 98% at 5 years and 96% at 10 years for the whole cohort. Peak O2, E/CO2 slope (ratio of minute ventilation to carbon dioxide production), and heart rate reserve during cardiopulmonary exercise testing predicted risk of early mortality when analyzed with logistic regression analysis; peak O2 emerged as the strongest predictor on multivariable analysis (odds ratio, 0.65 per 1 mL·kg−1·min−1; P=0.041). Conclusions— PVR after repair of tetralogy of Fallot has a low and improving mortality, with a low need for reintervention. Preoperative cardiopulmonary exercise testing predicts surgical outcome and should therefore be included in the routine assessment of these patients.

Use of the Bidirectional Glenn Procedure in the Presence of Forward Flow From the Ventricles to the Pulmonary Arteries

BACKGROUND Relative regression of the pulmonary arterial size has been reported after a conventional bidirectional Glenn procedure. Maintaining a supplemental pulmonary flow could be of surgical value unless the option also militates against the efficacy of the partial right heart bypass. METHODS AND RESULTS Twenty-seven patients considered unsuitable for a Fontan-type procedure underwent a bidirectional Glenn procedure in the presence of forward flow from the ventricles to the pulmonary arteries, the flow being maintained through the pulmonary trunk in 22 or a systemic-to-pulmonary shunt in 5. There was one surgical death due to atrioventricular valvular regurgitation. Subsequently, 9 patients have successfully undergone a total cavopulmonary connection 2.6 +/- 1.9 years after the initial procedure. Preoperative and postoperative catheterizations revealed changes in arterial oxygen saturation (75 +/- 11% compared with 83 +/- 7%, P < .001) and end-diastolic volumes of the systemic ventricles (from 238 +/- 92% to 188 +/- 97% of the expected normal volume, P < .01), whereas no difference was detected in the mean cross-sectional area of the right and left pulmonary arteries compared with the expected normal value for the right pulmonary artery (from 76 +/- 21% to 81 +/- 20%) or in the ventricular ejection fraction (from 53 +/- 8% to 50 +/- 14%). The relative regression or growth of the pulmonary arterial size was statistically related to the size of the channel for forward flow. CONCLUSIONS Maintenance of forward flow from the ventricle provides a feasible means, when performing a bidirectional Glenn procedure, of protecting against regression of pulmonary arterial size as well as off-loading the ventricles and improving arterial oxygen saturation.

Surgery for congential heart disease Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

To extend the indications for corrective operation in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, surgical procedures were done to unify the blood sources for pulmonary perfusion. Since December 1985, 50 patients have undergone unifocalization at ages from 2 months to 26 years with a mean of 6 +/- 7 years. In total, 84 staged unifocalization procedures and 5 other palliative procedures were done in 49 patients. These included several operative procedures: simple ligation of major aortopulmonary collateral arteries in 8; pulmonary angioplasty in 29 including reconstruction of the pulmonary arterial tree by direct anastomosis or interposition between the central pulmonary arteries and the intrapulmonary arteries; construction of artificial central pulmonary arteries with use of a xenograft pericardial tube graft in 36 with no native central pulmonary arteries detected; and construction of supplemental central pulmonary arteries also with use of a pericardial tube graft in 10. The pericardial tube graft, if used, was anastomosed to the intrapulmonary arteries on one end and connected to a prosthetic tube on the other end so as to perfuse the reconstructed pulmonary arteries. The anastomosis was made inside the lung through the divided interlobar fissure. Five patients died after operation among those undergoing these 89 preparative operative procedures. Deaths were related either to bleeding caused by anticoagulation therapy administered to prevent thrombosis within the xenograft pericardial tube graft used or to progressive congestive heart failure as a result of an excessive amount of pulmonary blood flow. Twenty-six patients have undergone intracardiac repair after previous unifocalization. In 16 patients the artificial central pulmonary arteries surgically constructed were connected to each other and then an external conduit was placed. In another patient, intracardiac repair and unifocalization could be concomitantly achieved via a median sternotomy. The right ventricle to left ventricle systolic pressure ratio immediately after intracardiac repair in 27 patients ranged from 0.24 to 0.91 with a mean of 0.54 +/- 0.17. One patient (4%) died shortly after intracardiac repair because of thrombosis within the pulmonary arteries. Postoperative catheterization showed that pulmonary vascular resistance was correlated significantly with the number of pulmonary vascular segments functioning rather than with the condition of the central pulmonary arteries. We conclude that surgical unifocalization is a feasible procedure before subsequent intracardiac repair, even in patients with critically hypoplastic or absent central pulmonary arteries.

Establishment of total cavopulmonary connection without use of cardiopulmonary bypass.

OBJECTIVE To minimize deleterious postoperative influences of cardiopulmonary bypass on the pulmonary circulation immediately after the Fontan type procedure, total cavopulmonary connection was achieved without use of cardiopulmonary bypass. METHODS Since April 1996, 15 patients including five patients with visceral heterotaxy, in whom no intracardiac procedure was needed, have undergone this operative maneuver. Age at operation ranged from 1.2 to 44.6 years. Construction of a systemic to pulmonary shunt had been previously employed in seven patients, banding of the pulmonary trunk in two patients, and the Norwood procedure in one patient. The superior caval vein was initially anastomosed to the pulmonary arteries in bidirectional fashion under temporary bypass from the superior caval vein to the atrium. The channel for draining the inferior caval vein was subsequently constructed with the aid of temporary bypass from the inferior caval vein to the atrium, using a Goretex tube in ten patients, using a pedicled autologous pericardial roll in four patients, and directly anastomosing the pulmonary trunk to the orifice of the inferior caval vein in one patient. In patients with visceral heterotaxy and an independent hepatic venous drainage, redirection of the blood flow via the caval vein as well as the hepatic vein could be successfully achieved by placing dual temporary bypasses into these veins. RESULTS Postoperative courses were excellent in all patients. Superior caval venous pressure was 11 +/- 2 mmHg at 12 h after the operation. No blood transfusion was needed in nine patients(60%). CONCLUSION This alternative operative procedure is undoubtedly attractive when establishing the Fontan circulation in patients undergoing no intracardiac maneuvers.

Result of surgical treatments in patients with coronary-arterial obstructive disease after Kawasaki disease.

OBJECTIVE To determine the efficacy of coronary artery bypass grafting (CABG) in young patients with coronary-arterial obstructive disease subsequent to Kawasaki disease. METHODS CABG was employed in 100 patients. Age at operation ranged from 1 to 23 years at a mean of 10+/-5 years. The number of bypass grafts placed was 1-5/patient (a mean of 1.7+/-0.8). The left internal-thoracic artery (ITA) was used as a graft in 99 patients; the right internal thoracic artery in 39, the gastroepiploic artery in nine and the saphenous vein in 21. RESULTS All patients survived the procedures. In the follow-up of 6.7+/-4.5 years, two patients died, one because of a traffic accident and the other due to sudden death. Considerable myocardial ischemia recurred postoperatively in 15, because of either obstruction of the bypass grafts or progression of other coronary-arterial obstructions. Of these, symptoms spontaneously regressed without interventional procedures in four, reoperation was indicated in four and catheter intervention was efficiently carried out in the remaining seven. Another two patients had episodes of critical ventricular arrhythmia; one of them with severe left ventricular dysfunction subsequently underwent cardiac transplantation. The patency rates of the arterial grafts were 94, 82 and 78% at 1, 5 and 10 years, respectively, and this was higher than that of the venous grafts (82, 63 and 36%, respectively). Strenuous exercise is currently prohibited in 15 patients, while the remaining 83 patients are doing well with no obvious restriction in their daily lives. CONCLUSION Collaborating with catheter interventions, CABG using the arterial grafts can provide attractive results in patients with obstructive coronary arteries associated with Kawasaki disease.