Efficacy and safety of torasemide in children with heart failure.

OBJECTIVE: To examine the efficacy and safety of torasemide in children with chronic heart failure (HF). METHODS: 102 children with chronic HF who had received oral torasemide were analysed. Of these, 62 (de novo group) were newly diagnosed as having HF and were given torasemide as a diuretic. The remaining 40 (replacement group) had been given furosemide for >3 months before the study, and furosemide was then replaced with torasemide. Clinical signs and symptoms of HF (assessed as the HF index), humoral factors and serum potassium concentrations before torasemide treatment were compared with those obtained 3-4 weeks after torasemide treatment. Patients were also monitored for adverse effects. RESULTS: In the de novo group, torasemide significantly improved the HF index with concomitant improvement in plasma brain natriuretic peptide concentration (median (interquartile range) 52 (51) vs 43 (49) pg/ml). In a randomly selected group of 25 de novo patients with ventricular septal defect, echocardiography showed that torasemide significantly improved left ventricular geometry and function. In the replacement group, brain natriuretic peptide concentrations were also significantly decreased from 50 (104) to 45 (71) pg/ml after substitution of torasemide, but the HF index showed only a tendency for improvement (p = 0.07). Torasemide also had a potassium-sparing effect (de novo group, no change in potassium concentration; replacement group, significant increase from 4.2 (0.5) to 4.3 (0.5) mEq/l), and caused a significant rise in serum aldosterone concentration, consistent with the anti-aldosterone effect of this drug. Serum concentrations of sodium and uric acid had not changed after torasemide treatment, and there were no serious adverse events that necessitated drug withdrawal. CONCLUSION: Torasemide can be safely used, and appears to be effective for treatment of HF in children. Future clinical trials are warranted to verify the present results.

Arterial haemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular after load and aortic dilatation.

BACKGROUND: Recent histological studies of the aortic wall of patients with tetralogy of Fallot (TOF) have shown massive degeneration of the tunica media of the aorta. Such changes in arterial wall structure may significantly alter arterial wall mechanical properties, and thus cause abnormal arterial haemodynamics. OBJECTIVE: To test the hypothesis that after repair of TOF, there are abnormal arterial haemodynamics which are associated with aortic dilatation and which increased after load on the left ventricle. METHODS AND RESULTS: The subjects comprised 38 patients who had undergone complete repair of TOF, and 55 control subjects. Systemic arterial haemodynamics were investigated by measuring aortic input impedance during cardiac catheterisation. The patients with TOF had significantly higher characteristic impedance (158 (43) dyne x s x cm(-5) x m(2) vs 105 (49) dyne x s x cm(-5) x m(2)) and pulse wave velocity (561 (139) cm/s vs 417 (91) cm/s) and significantly lower total peripheral arterial compliance (0.93 (0.39) ml/mm Hg/m(2) vs 1.24 (0.58) ml/mm Hg/m(2)) than the controls (for all three variables, p<0.01 vs controls), suggesting that central and peripheral arterial wall stiffness are increased after TOF repair. Additionally, patients with TOF had significantly higher arterial wave reflection than the controls (reflection coefficient: 0.21 (0.12) vs 0.16 (0.06)). These abnormalities in patients with TOF increased the pulsatile load on the left ventricle and significantly contributed to decreased cardiac output, even when right ventricular function was taken into account by multivariate regression analysis. The increase in aortic wall stiffness was closely associated with the increase in aortic root diameter. CONCLUSION: These results indicating abnormal arterial haemodynamics after TOF repair highlight the importance of regular monitoring of the systemic arterial bed and potentially relevant cardiovascular events in long-term follow-up of TOF.

[Coronary malperfusion due to acute type A aortic dissection; surgical strategy and results].

Coronary malperfusion due to acute type A aortic dissection (DAA) is a lethal complication. It is especially difficult to rescue the patients with left coronary malperfusion because of acute global myocardial infarction (AMI), even with successful surgical treatments, including the replacement of the ascending aorta and coronary artery bypass grafting (CABG). We review our experience and illustrate our approach to these critically ill patients. In addition, we classify the mechanism of malperfusion into 4 types based upon perioperative findings and discuss surgical management indivisually. From January 1990 to April 2005, a total of 260 patients were operated for DAA in our institution. Twenty (7.7%) patients, 11 men and 9 women were suffering from coronary malperfusion due to DAA. The mean age was 55 (range 28-72) years. The right coronary artery was involved in 9 patients, and the left in 11. All procedures such as graft replacement and CABG were done on an emergent or urgent basis. Hospital mortality rate of right coronary malperfusion was 22% (2/9 patients), and that related to left coronary malperfusion was 5/11 (45%). Assisting device was required in 9 cases, veno-arterial bypass (VAB) in 6 cases, left ventricular assist system (LVAS) in 1, left heart bypass (LHB) in 1, LHB+right heart bypass (RHB) in 1. We lost all patients using VAB. Only 3 patients supported with strong assist device survived. Aggressive myocardial resuscitation and early operation are the key factors in the management of these critically ill patients. But once severe myocardial infarction occurs, V-A bypass (percutaneous cardiopulmonary support) is useless in treating patients with DAA who develop severe heart failure. We recommend to implant stronger assist device including LVAS immediately before exacerbation of multiple organ failure. In conclusion, surgical management is not easy for emergency patients with DAA in association with myocardial ischemia. However, reasonable surgical results can be obtained with supplemental CABG and strong mechanical support of the left ventricle.

[Showing no increase of C-reactive protein and developing non-bacterial median chest wound dehiscence after coronary artery bypass grafting].

A 72-year-old male underwent coronary artery bypass grafting for 3 vessels disease. After the operation, the peak C-reactive protein (CRP) level was 0.85 mg/dl and CRP levels stayed very low. Forty days after the operation the patient developed a progressive median chest wound dehiscence but the bacterial cultures were negative. In spite of conventional therapies such as debridement of necrotic tissue or irrigation, the wound granulation was underdeveloped. After 6 months an epidermis had developed to cover the wound.

[The role of staged Fontan operation on early and long-term outcome].

Of 185 patients with single ventricle physiology, 59 patients who underwent Fontan type operations between April 1970 and May 2002 served as subjects. Subjects displayed a median age of 5.2 years and a median body weight of 11.4 kg. In the first 22 years (group 1), 34 patients underwent concomitant right atrium-pulmonary artery (RA-PA) anastomosis, Björk procedure and total cavopulmonary connection (TCPC), while in the last 8 years (group 2), 25 patients underwent staged TCPC, where bidirectional cavopulmonary shunt (BCPS) and obliteration of additional pulmonary blood flow was performed previously. Four cases of early death (group 1:4 patients, group 2:0 patient) and 12 cases of late death (group 1:11 patients, group 2:1 patient) were encountered. Early mortality was 6.8% (group 1:12%, group 2:0%, p = 0.10) and late mortality was 21%. The 5-year survival rate was 85.2 +/- 4.9% (group 1:80.0 +/- 6.8%, group 2:93.8 +/- 6.1%, p = NS), and the 10-year survival rate was 79.6 +/- 6.0%. Staged TCPC, precedent BCPS with obliteration of additional pulmonary blood flow, seems to be beneficial for accurate patient selection for Fontan candidate.

[A case of atypical aortic coarctation with mitral and aortic regurgitation and ascending aortic dilatation].

A 67-year-old female presented with dyspnea on exertion as a chief complaint. Diagnosed as having severe mitral regurgitation, aortic regurgitation, dilatation of the ascending aorta and atypical coarctation due to aortitis syndrome, she underwent mitral valve replacement, aortic valve replacement, ascending aorta and hemiarch replacement and ascending aorta-abdominal aorta extraanatomical bypass in one stage. Pathologically, typical findings of aortitis syndrome were not observed in the wall of the ascending aorta and aortic valve, but cystic medionecrosis was noted in the wall of the ascending aorta. Follow-up observations are needed for the remaining aortic wall.

[Current trends in heart surgery for the treatment of congenital anomalies: tetralogy of Fallot and its variants].

The surgical repair of tetralogy of Fallot is one of the most common procedures to correct congenital cardiac anomalies. However, controversy remains regarding the benefits and drawbacks of early primary repair. The management of patients with pulmonary atresia has become more aggressive in recent years, i.e., earlier primary repair with complete unifocalization is performed even if they have associated major aortopulmonary collateral arteries. On the other hand, the durability of small-sized conduits is unsatisfactory, and the rate of freedom from reoperation for those who received conduits in infancy is still low. Ventricular arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot are disastrous complications in adult survivors, but it remains difficult to predict which patients will experience such complications. Sustained ventricular tachycardia with pulmonary regurgitation is the predominant hemodynamic lesion. Achieving complete relief of the pressure gradient in the right ventricular outflow tract with no pulmonary regurgitation is the ultimate goal of surgical treatment of tetralogy of Fallot and its variants.

Optimal position of atrial epicardial leads for temporary pacing in infants after cardiac surgery.

BACKGROUND: The atrial epicardial wall of pediatric patients was topographically assessed to identify the optimal position for temporary atrial pacing. METHODS: Unipolar electrodes were fixed at the cephalic wall between the right and left atrial appendages, the interatrial groove, and the right atrial appendage of fifteen pediatric patients who underwent definitive surgical repair. The performance of the three electrodes in terms of pacing patterns and six combinations of bipolar pacing were evaluated in terms of the pacing threshold, P-wave amplitude, slew rate, and lead impedance. RESULTS: Unipolar pacing of the right atrial appendage showed a significantly higher threshold than the other groups. A bipolar configuration of the cephalic atrial wall and interatrial groove had a significantly higher P-wave amplitude than groups without the electrode at the cephalic atrial wall, and a significantly higher slew rate than a unipolar configuration of the atrial appendage. CONCLUSIONS: Bipolar pacing with the negative electrode at the cephalic atrial wall and the indifferent electrode at the interatrial groove is the most efficient method for pediatric patients.

Left atrial appendage insertion for right ventricular outflow tract reconstruction.

BACKGROUND: The left atrial appendage (LAA) may serve as an alternative to the pulmonary arterial wall for right ventricular outflow tract (RVOT) reconstruction without an extracardiac conduit. METHODS: Five consecutive patients with pulmonary atresia or severe stenosis underwent corrective (n = 4) or palliative (n = 1) RVOT reconstruction using an LAA insertion. Surgery was performed to treat tetralogy of Fallot, double-outlet right ventricle, or transposition of the great arteries. By inserting the LAA into the obstructed portion, the width of the posterior wall of the RVOT was 20 mm or more. The anterior half of the RVOT was then augmented with pericardial patch. RESULTS: There were no early or late postoperative deaths, and no major complications (arrhythmias, thrombo-embolic episodes, infective endocarditis, need for reoperation). The postrepair systolic right ventricular-to-systemic arterial pressure ratio was 0.61 +/- 0.26. Color Doppler flow mapping revealed that the reconstructed RVOT was nonobstructive and had nonturbulent flow. No thrombus or pseudoneointimal formation was observed in the RVOT. CONCLUSIONS: LAA insertion in the RVOT is an effective alternative to, or adjunct of, direct anastomosis. It offers several advantages, including fewer early and midterm complications and avoiding the use of an extracardiac conduit.

Placement of interatrial patch suture lines in atrioventricular canal defect repair.

BACKGROUND: The placement of the suture line for interatrial patches in complete and incomplete atrioventricular canal defect repairs varies from surgeon to surgeon despite established anatomic knowledge of the atrioventricular conduction system. This study describes our technique for it and reviews early and long-term outcomes. METHODS: Between 1980 and 1999, 64 infants and children underwent repair of either complete (n=39) or incomplete (n=25) atrioventricular canal defects. Thirty-four of the children (53.1%) had Down's syndrome. The suture line for the interatrial patch originated on either the artificial or native ventricular septal crest and continued leftward above the annulus of the left inferior leaflet of the atrioventricular valve at the posteroinferior corner. All stitches were placed in a horizontal mattress or U-shaped fashion. RESULTS: The operative survival rate was 94% (4 early deaths) and the overall survival rate was 85% (6 late deaths). Atrioventricular heart blocks occurred in none of the patients. Although left-sided atrioventricular function significantly improved with repair, two patients (3.1%) required reoperation for valve replacement because of residual or recurrent insufficiency. CONCLUSIONS: This suture technique for interatrial patches is straightforward and results in a low incidence of heart block and a low re-operation rate for left atrioventricular valve insufficiency.

Omental flap for mediastinitis after median sternotomy in asplenia syndrome and gut malrotation.

We describe a 12-year-old patient with asplenia syndrome and gut malrotation who, after an interim step before a modified Fontan operation, developed life-threatening mediastinitis. A flap of the omentum arising from the transverse colon, longitudinally located in the left of the abdomen, was created and transferred to the mediastinum after a division of the arterial arcade at its most caudal extent. Her postoperative course was uneventful, and 12 months later, the modified Fontan operation was successfully completed. Although visceral heterotaxy results in an omental deformation, it does not preclude the use of an omental flap.

Factors influencing arterial oxygenation early after bidirectional cavopulmonary shunt without additional sources of pulmonary blood flow.

OBJECTIVES: Although the arterial oxygen saturation after bidirectional cavopulmonary shunting should theoretically be homogeneous if additional pulmonary flow is obliterated, the arterial oxygen saturation has been found to vary in clinical practice. Knowledge of the preoperative and operative determinants of arterial oxygen saturation early after bidirectional cavopulmonary shunting may lead to a better understanding of this unique physiology. METHODS: Thirty-five patients who underwent bidirectional cavopulmonary shunting with obliteration of additional pulmonary flow were included in this study. The arterial oxygen saturation was determined at the 5 time points over a 48-hour period. Multivariable regression analysis was used to identify the independent predictors of the arterial oxygen saturation. RESULTS: No significant interval changes occurred in the arterial oxygen saturation during the 48 hours after bidirectional cavopulmonary shunting, which ranged from 61.6% to 95.6%. There was a significant inverse correlation between the postoperative superior vena cava pressure and the arterial oxygen saturation (P =.003). A low arterial oxygen saturation early after bidirectional cavopulmonary shunting was a predictor of mortality or exclusion from univentricular repair within 24 months (P =.012, odds ratio = 1.14). Of 11 factors identified by univariable analysis, multiple regression analysis indicated that age less than 8 months at the time of shunting (P <.0001) and ventricular volume overload (P =. 002) predicted a lower arterial oxygen saturation after bidirectional cavopulmonary shunting. CONCLUSIONS: Even without additional sources of pulmonary blood flow, several preoperative factors, including younger age and severe ventricular volume overload, predicted a decrease in the arterial oxygen saturation early after bidirectional cavopulmonary shunting. This, in turn, predicted poor outcome during 2 years of follow-up.

Primary repair of complete transposition of the great arteries with complete atrioventricular septal defect.

We successfully corrected complete transposition of the great arteries associated with complete atrioventricular septal defect in a 50-day-old infant in concomitant arterial switch operation and two-patch repair. The combination of these 2 complex anomalies is very rare, and primary anatomical repair is feasible in such patients.

Aortoventriculoplasty in patients with aortic malposition.

Aortoventriculoplasty was applied successfully for recurrent combined subvalvular and valvular aortic obstruction that developed after intraventricular baffle repair of a cardiac anomaly in 2 patients with associated anterior malposition of the aorta. A single baffle for the left ventricular tunnel was also used for aortic annulus augmentation.

Hemashield implantation in young patients with congenital cardiovascular lesions.

BACKGROUND: There is substantial controversy regarding the use of Hemashield in young patients. METHODS: Twenty-one consecutive patients younger than 20 years of age with a variety of congenital cardiovascular lesions underwent surgical procedures using a Hemashield woven graft. Hemashield was used for reconstruction of the aortic wall (n = 16), ventricular septum (n = 10), and right ventricular free wall or pulmonary artery (n = 6). RESULTS: A sterile inflammatory reaction was observed including high fever, increased white cell count, and elevated plasma C-reactive protein concentration for up to 4 weeks after implantation. Multivariable analysis identified the use of Hemashield in the right ventricular free wall or pulmonary artery as an incremental risk factor for elevation of plasma C-reactive protein concentration during the first 3 weeks after implantation (p = 0.002). There were no midterm complications including restenosis of the grafts in the right ventricular outflow tract. CONCLUSIONS: Hemashield can be used in a variety of situations for reconstruction of congenital cardiovascular lesions in young patients. Impregnated collagen can cause a significant systemic inflammatory reaction for several weeks after implantation, especially when used in the low-pressure right heart.

Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt.

BACKGROUND: Multiple systemic-to-pulmonary artery shunts often represent a deviation from the initial management strategy for cyanotic congenital heart disease. This study analysed the outcome in patients undergoing a second shunt. METHODS: Between 1965 and 1998, 80 patients required a second shunt. Patient age ranged from 11 days to 11.2 years at the initial shunt, and 4 months to 25.6 years at the second shunt. The interval between shunts ranged from 11 days to 19.6 years. RESULTS: The patient survival rates at 1, 6, 12, 60 and 120 months following the second shunt were 95.0 +/- 2.4%, 92.5+/-3.0%, 87.4+/-3.7%. 82.4+/-4.5% and 74.8+/-5.8%, respectively. Although excess pulmonary flow was not encountered after the second shunt, mode and incidence of morbidity were otherwise similar to the first one. Between 9 months and 7.5 years after the second shunt, 24 patients underwent successful definitive repair. Multivariable analysis identified pulmonary atresia (p=0.027) and a short (<1 year) interval between the two shunts (p=0.034) as the independent risk factors for long-term shunt failure. Single ventricle physiology (p=0.002) and a central approach for the second shunt (p=0.016) were independent risk factors for lack of application, or failure of intracardiac definitive repair. CONCLUSIONS: A significant limitation in longevity and quality of life is common in patients requiring a second shunt, especially those associated with pulmonary atresia, the single ventricle physiology heart, or ungraftably hypoplastic contralateral branch pulmonary artery to the first shunt. Since excess pulmonary blood flow leading to congenital heart failure and/or ventricular diastolic dysfunction is unlikely, all patients who preclude definitive repair due to decreased pulmonary blood flow even after the first shunt should be shunted again.

Modified ultrafiltration improves carbon dioxide removal after cardiopulmonary bypass in infants.

Little is known about the role of modified ultrafiltration in ameliorating the adverse effects of the cardiopulmonary bypass on pulmonary function in infants. Twenty-nine nonrandomized consecutive infants (<12 months of age) who underwent unrestrictive ventricular septal defect closure between 1995 and 1998 were included in this study. Down's syndrome was associated in 9 patients. The actual ventilator settings were highly homogeneous among all patients and each time point in the study. Fourteen infants received modified ultrafiltration after the discontinuation of cardiopulmonary bypass. Fifteen untreated patients served as the control group. Correlates of cardiac and pulmonary functions for both groups were compared. The arterial carbon dioxide tension in the experimental group was significantly lower than in the control group from 20 to 240 min after bypass. Arterial oxygenation and pulmonary arterial pressure were similar in the 2 groups. Modified ultrafiltration improves carbon dioxide removal after cardiopulmonary bypass in infants. This may potentially convey a beneficial impact on hemodynamics.

Surgical management of the straddling mitral valve in the biventricular heart.

BACKGROUND: The straddling mitral valve in the biventricular heart is a rare condition that may complicate biventricular repair. METHODS: Treatment and outcomes in 5 consecutive patients who underwent primary repair between 1992 and 1997 were reviewed. Their ages at repair ranged from 2 months to 8 years. Three patients had a double-outlet right ventricle with a subaortic (n = 2) or subpulmonary (n = 1) ventricular septal defect. Two patients had transposition of the great arteries (S,D,D), a ventricular septal defect, and left ventricular outflow tract obstruction. The attachments of the papillary muscles of the straddling mitral valves were located on the right ventricular aspect of the ventricular septum. Four patients underwent baffle partitioning of the ventricular cavity. The baffle suture line was used to secure the chordae tendineae crossing the ventricular septal defect, or was intentionally omitted at the papillary muscle. The right ventricular outflow tract was reconstructed with patch augmentation, an extracardiac conduit, or an arterial switch operation. One patient with transposition who had a giant papillary muscle to the straddling mitral valve associated with abnormal insertion of the tricuspid valve on the conal septum underwent univentricular repair. RESULTS: There were no early or late postoperative deaths. There was no mitral valve dysfunction, left ventricular outflow tract obstruction, or heart block in the 4 patients who underwent biventricular repair. CONCLUSIONS: Although there are several exceptional situations in which ventricular partitioning may result in early and late complications, a straddling mitral valve does not preclude biventricular repair.

Surgical angioplasty of left main coronary stenosis complicating supravalvular aortic stenosis.

We successfully treated obstruction of the main coronary artery, not aortic valve leaflet adhesion to the intimal shelf, complicating supravalvular aortic stenosis by modifying the Brom aortoplasty. An autologous pericardial patch was used to enlarge the left main coronary artery as well as the stenotic aorta. This modification allows simple and effective restoration of coronary blood flow, while maintaining the Brom procedure's merit of achieving anatomic geometry of the aortic root in such patients.