A case of malignant pheochromocytoma with Holt-Oram syndrome.

A 23-year-old female patient with malignant pheochromocytoma was admitted to the Tokyo Women's Medical University. The patient had been clinically diagnosed with Holt-Oram syndrome at birth. Since she had complex congenital heart disease, chronic heart failure, and severe hypoxia, the risk surrounding surgery to remove the primary tumor was predicted to be very high, and subsequently, chemotherapy was performed. The patient was not able to continue chemotherapy due to adverse effects. However, for one year, both her hypertension and catecholamine-dependent symptoms were well controlled by an alpha-adrenergic and beta-adrenergic receptor blockade, although the patient did experience high plasma norepinephrine levels. To our knowledge, this is the first report of a patient with the combination of malignant pheochromocytoma and Holt-Oram syndrome. A correlation between chronic hypoxia and pheochromocytoma has been reported. This instructive case reminds us to consider the possibility of pheochromocytoma with congenital heart disease when these types of unexpected or unusual symptoms are encountered.

Mortality of pulmonary artery banding in the current era: recent mortality of PA banding.

BACKGROUND: The mortality of pulmonary artery banding improved significantly in the 1980s. However, we lack information on this procedure in the current era. METHODS: The results of pulmonary artery banding in 365 patients who had operations between 1966 and 2001 were reviewed. The patients were divided into three groups: (1) group 1 patients who had operations between 1966 and 1979, (2) group 2 patients who had operations between 1980 and 1989, and (3) group 3 patients who had operations between 1990 and 2001. RESULTS: Significantly younger and smaller patients have been operated on recently (mean age: group 1, 169.0 +/- 40 days; group 2, 101.8 +/- 11 days; and group 3, 69.7 +/- 8.9 days; and mean weight: 4.6 +/- 0.1, 4.1 +/- 0.1, and 3.2 +/- 0.1 kg, respectively). A decrease was found in the number of simple cardiac anomalies, such as isolated ventricular septal defects. The early mortality in the three groups was 38.3% for group 1 (65 of 187), 13.5% for group 2 (15 of 111), and 13.8% for group 3 (12 of 87). Although the mortality did not vary significantly between groups 2 and 3, it improved over time in patients weighing less than 3 kg. Multivariate analysis of group 3 demonstrated that no isolated variable, including sex, weight, and diagnosis was a significant risk factor. CONCLUSIONS: Despite the advances in perioperative management, we found no improvement in the early mortality of pulmonary artery banding during the last decade. These results will support the preference for primary repair of intracardiac anomalies in small infants. However, this operation can now be performed with the same risk even in smaller patients. We believe that pulmonary artery banding has a role in the treatment of congenital cardiac anomalies.

As originally published in 1996. Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan patients. Updated in 2002.

BACKGROUND: A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation. METHODS: Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 +/- 257 days). RESULTS: Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p = 0.01). Pumonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly from 1.8 +/- 1.1 to 0.7 +/- 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them. CONCLUSIONS: The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.

Age-associated changes in arterial elastic properties in children.

UNLABELLED: Age-associated changes in pulsatile components of the arterial system in normal children have not previously been studied. The purpose of the present study was to establish reference ranges for age-associated changes in arterial pulsatile properties in children. Arterial compliance (C(A)) and stiffness of the proximal aorta were estimated from data obtained by cardiac catheterisation in 112 paediatric patients (age range, 6 months to 20 years) who were considered to have normal systemic vascular beds. C(A) was calculated by the area method, using ascending aortic pressure tracings. Aortic wall stiffness was evaluated using the pressure-strain elastic modulus (Ep). C(A) significantly increased with age (C(A) =0.06 5 x age +0.86, r=0.71, P<0.001). However, the value of C(A)/BSA, which represents normalisation of C(A) to body surface area (BSA) (to eliminate factors associated with development of arterial size), significantly decreased with age and declined fastest during the first several years of life, suggesting an increase in arterial wall stiffness with increasing age in children. Ep also significantly increased with age (Ep =9.8 x age +177, r=0.50, P<0.001). CONCLUSION: these findings suggest that both peripheral and proximal arterial wall distensibility in children decline after birth, although total arterial buffering capacity increases. The present results provide the first quantification of in vivo age-associated changes in arterial elastic properties in children, and are potentially useful as a reference for future studies of patients with diseases that involve the vascular system.

Transcatheter closure of atrial septal defects with the Amplatzer septal occluder--a Japanese clinical trial.

This study reports the results of a Japanese clinical trial of transcatheter closure of atrial septal defects (ASD) using the Amplatzer septal occluder (ASO). Thirty-five patients with secundum ASD underwent transcatheter closure using the ASO at a median age of 12.9 years (range, 3.2-29.2 years) and a median weight of 39.2kg (range, 11.6-65.1 kg). The ASO was successfully implanted in 34 patients. The mean ASD diameter of the 34 patients measured by transesophageal echocardiography was 11.7 +/- 4.2mm (range, 5.0-20.8mm) and the mean balloon stretched diameter was 16.8 +/- 4.2 mm (range, 9-25 mm). The mean ASO size was 16.9 +/- 4.3 mm (range, 9-26mm). Complete closure rate at 1 day and 1 year after closure was 91% and 97%, respectively. One patient developed a transient second-degree atrioventricular block during the implantation procedure. No other complications occurred. Transcatheter closure of ASD using the ASO is effective and safe.

Dilatable banding of a Blalock-Taussig shunt.

Dilatable banding has been used in various situations. Sometimes Blalock-Taussig shunt banding is performed to prevent pulmonary overcirculation. Recently several reports have described dilatable pulmonary artery banding. We modified these methods for flow control of a Blalock-Taussig shunt. We report the case of a neonate with truncus arteriosus in which this technique was used.

Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts.

BACKGROUND: Recent studies have indicated that there are inherent limitations associated with Fontan physiology. However, there have been no quantitative analyses of the effects of right heart bypass on ventricular afterload, hydraulic power, and resultant overall hemodynamics. Methods and Results- During routine cardiac catheterization, aortic impedance and ventricular hydraulic power were determined, both at rest and under increased ventricular work induced by dobutamine, in 17 patients with Fontan circulation, 15 patients with a single ventricle whose pulmonary circulation was maintained only by Blalock-Taussig shunts, and 13 patients who had normal 2-ventricle circulation. Both vascular resistance (nonpulsatile load on the ventricle) and pulsatile components of ventricular afterload (represented by low-frequency impedance) were significantly higher in the Fontan group than in the other groups (P<0.01), and this was associated with decreased cardiac output in the Fontan patients. In addition, hydraulic power cost per unit forward flow was 40% lower in the 2-ventricle circulation than in the single-ventricle circulation, suggesting lower ventricular efficiency in single-ventricle circulation attributable to the lack of a pulmonary ventricle. Furthermore, in the Fontan group, beta-adrenergic reserve was markedly decreased because of a limited preload reserve. CONCLUSIONS: Fontan physiology is associated with disadvantageous ventricular power and afterload profiles and has limited ventricular reserve capacity. Thus, to improve the long-term prognosis of patients after Fontan surgery, future research should be conducted into medical interventions that can overcome these limitations inherent in Fontan circulation.

Mortality and risk factors for late deaths in tetralogy of Fallot: the Japanese Nationwide Multicentric Survey.

OBJECTIVES: We have compared mortality and risk factors for late deaths in patients with tetralogy of Fallot undergoing surgical repair in 1972 and 1982 in a Japanese multicentric study, examining in particular the impact of time of repair. BACKGROUND: There is limited information on the effect that time of repair, and our constantly changing approach to it, has on late outcome in repaired tetralogy of Fallot. METHODS: We analysed the Japanese registry of deaths occurring after surgical repair of tetralogy of Fallot. We studied two postoperative 1-year cohorts of survivors of surgery performed in 12 centers. Of the patients, 122, aged 29+/-12 years, had undergone repair in 1972, their age at repair being 9.6 years. An additional 186 patients, aged 23+/-8.7 years, had been repaired in 1982 at the age of 7.7 years. RESULTS: Annual mortality, as judged per 100,000 population of patients with tetralogy of Fallot, declined from 0.387 in 1972 to 0.196 in 1982. Significant differences were deaths following surgery (27% vs. 13%, p < 0.001), patching of the subpulmonary outflow tract (48% vs. 89%, p < 0.001), and transjunctional patching (13% vs. 63%, p < 0.001). Late death was observed in 6 vs. 3 patients (9/308, 2.9%). The actuarial rate of survival calculated over 14 years was 97% vs. 98%. Reoperation was performed in 5 vs. 9 patients (14/308, 4.5%). Risk factors for late death were age at repair (p = 0.01), and history of reoperation (p < 0.001). Transjunctional patching (p = 0.01) proved to be associated with late mortality only in patients repaired in 1972. CONCLUSIONS: Late survival was excellent, with a low incidence of reoperations in both groups of patients. The era of repair has a big influence on total and operative mortality, but has only a small impact on late mortality. Ongoing analysis of follow-up will possibly reveal subsequent changes with time.