Ventricular fibrogenesis activity assessed by serum levels of procollagen type III N-terminal amino peptide during the staged Fontan procedure.

OBJECTIVE: We tested the hypotheses that volume overload and cyanosis observed in the pre-Fontan single ventricular circulation are associated with increased ventricular fibrogenesis, that the Fontan procedure helps to reduce fibrogenesis, and that persistently increased fibrogenesis in the Fontan ventricle is associated with ventricular diastolic dysfunction. METHODS: Levels of serum amino-terminal procollagen type III, a marker of tissue fibrogenesis, were measured in 172 patients with single ventricle circulation and 149 controls. Patients were divided into 3 groups according to surgical stage: 59 patients after Blalock-Taussig shunt or pulmonary banding, 60 patients after Glenn surgery (Glenn group), and 53 patients after Fontan surgery (Fontan group). RESULTS: Serum amino-terminal procollagen type III levels were significantly higher among the 3 single ventricle groups than among control patients, but decreased with each surgical stage (0.604, 0.176, 0.143, and 0.073 U/mL, for Blalock-Taussig shunt or pulmonary banding, Glenn, Fontan, and controls, respectively). Severity of volume load and cyanosis were independent determinants of increased amino-terminal procollagen type III levels in patients before Fontan surgery, and persistently increased amino-terminal procollagen type III after Fontan surgery was associated with ventricular diastolic stiffening (r = 0.494, P = .009). Data also indicated close associations between amino-terminal procollagen type III levels and activation of the renin-angiotensin-aldosterone system, suggesting potential involvement of this hormonal system in the increased fibrogenesis after Fontan surgery. CONCLUSIONS: These results suggest that serum amino-terminal procollagen type III may provide important diagnostic information on myocardial fibrosis in patients with single ventricle circulation and raise the possibility that ventricular fibrogenesis may be a potential therapeutic target in this population.

Stent Implantation for Effective Treatment of Refractory Chylothorax due to Superior Vena Cava Obstruction as a Complication of Congenital Cardiac Surgery.

Chylothorax is a serious complication of congenital cardiac surgery and is significantly associated with increased morbidity and mortality. Central venous obstruction, which is often related to the insertion of central venous catheters for postoperative management, is known to be an important risk factor for treatment failure and mortality associated with this condition. We present the case of a 6-month-old girl with refractory chylothorax after surgical repair of tetralogy of Fallot. The chylous drainage continued for more than 2 months despite maximal conservative therapy (water restriction, total parenteral nutrition, and infusion of somatostatin and steroid) and surgical ligation of the thoracic duct. Subsequently, we observed stenosis of the superior vena cava (SVC) caused by large thrombi possibly associated with the prolonged use of central venous catheter placed in the internal jugular vein. Because transcatheter balloon dilation failed to relieve the stenosis, we performed stent implantation for the SVC and innominate vein. After the procedure, chylous drainage dramatically reduced, and the patient was discharged from the hospital. In conclusion, central venous obstruction due to thrombosis should be routinely examined when chylothorax is diagnosed and is resistant to conservative therapy after congenital heart surgery. Stent implantation can effectively relieve the venous obstruction and thus be a life-saving treatment option for this difficult condition.

Findings in the pulmonary vascular bed in the remote phase after Kawasaki disease.

Kawasaki disease (KD) is a form of systemic vasculitis that causes chronic changes in arterial walls, including pulmonary arteries. The aim of this study was to test the hypothesis that pulmonary arterial wall properties and hemodynamics are abnormal after the resolution of KD. Pulmonary arterial input impedance was measured during cardiac catheterization (4.8 ± 4.5 years after disease onset) in 47 consecutive patients (mean age 7.8 ± 5.7 years) with KD and coronary artery lesions (CALs) in the acute phase and 42 control patients (mean age 6.7 ± 4.6 years). Patients with KD were subdivided into 2 groups: 28 with persistent CALs and 19 with regressed CALs. There were no significant differences in characteristic impedance and peripheral vascular resistance between patients with KD and controls. Compared with controls, patients with persistent CALs had significantly lower pulmonary arterial compliance, suggesting increased wall stiffness of the peripheral pulmonary vascular bed (p <0.05, analysis of variance). Patients with persistent CALs also exhibited increased wave reflection compared with other groups (p <0.05, analysis of variance). In conclusion, unlike patients with regressed CALs, patients with persistent CALs have abnormal mechanical properties and hemodynamics of the pulmonary artery after KD. Together with previous reports of abnormal properties of coronary and systemic arteries, these data suggest that KD vasculitis causes chronic changes in arterial wall properties in the entire arterial system to varying degrees and extent. The fate of these abnormalities in the pulmonary bed and other arterial systems and their potential adverse effects must be monitored in long-term follow-up.

Late clinical manifestations of mitral valve disease and severe pulmonary hypertension in a patient diagnosed with premature closure of foramen ovale during fetal life.

BACKGROUND: The patency of foramen ovale (FO) in fetal circulation is very important, and premature closure of FO could be associated with several pathological conditions. METHODS: We report a patient in whom premature closure of FO in fetal life was associated with late clinical onset of mitral valve stenosis and subsequent development of irreversible pulmonary hypertension (PH). RESULTS: The patient showed persistent PH after birth, which completely regressed at the age of 8 months. However, the patient developed heart failure due to mitral valve lesions (hammock valve) at the age of 11 months and underwent artificial valve replacement. The patient subsequently developed severe PH, which was refractory to anti-PH therapy with sildenafil and bosentan in addition to home oxygen. CONCLUSIONS: This case illustrates that mitral stenosis can be overlooked during early neonatal life, and thus emphasizes the need for close follow-up for potential existence of mitral stenosis and later clinical manifestation in patients with premature FO closure even when initial careful examination of the mitral valves does not indicate any abnormalities. In addition, premature closure of FO could cause pulmonary vascular disease, which may lead to later development of irreversible PH.

Effects of stent implantation for peripheral pulmonary artery stenosis on pulmonary vascular hemodynamics and right ventricular function in a patient with repaired tetralogy of Fallot.

Stent implantation is an effective alternative to surgery and balloon angioplasty for the treatment of stenotic vascular lesions. However, there is concern about the hemodynamic consequences related to the loss of vascular elasticity caused by the non-compliant property of the implanted stent. Here we report data for pulmonary vascular impedance and right ventricular performance after implantation of balloon-expanded stents for peripheral pulmonary stenosis in a 13-year-old girl with repaired pulmonary atresia and ventricular septal defect. Stent implantation reduced total vascular resistance, low-frequency impedance and wave reflection, and improved right ventricular contractile performance measured by the pressure-area relationship. However, pulmonary vascular wall stiffness remained elevated after stenting, and it was suggested that stent implantation resulted in a net increase in vascular wall stiffness. The long-term effects of implanted stents on vascular and ventricular hemodynamics need to be clarified.

Usefulness of respiratory variation of inferior vena cava diameter for estimation of elevated central venous pressure in children with cardiovascular disease.

BACKGROUND: The purpose of the present study was to determine the relationship of inferior vena cava diameter (IVCD) and its respirophasic variation (IVC collapsibility index: IVCCI) with central venous pressure (CVP), and thereby to provide reference cut-offs for such IVC parameters to estimate elevation in CVP in pediatric patients with cardiovascular disease. METHODS AND RESULTS: The study involved consecutive pediatric patients (n = 118) with various heart diseases who either had a central venous catheter in the cardiac intensive care unit or underwent cardiac catheterization. The maximum (IVCD(max)) and minimum (IVCD(min)) diameters of IVC were measured on ultrasound simultaneously with measurements of mean CVP. IVCD(max), IVCD(min) and IVCCI correlated significantly with CVP (R² = 0.26, 0.47 and 0.41, respectively) in spontaneously breathing patients, but not in mechanically ventilated patients. Receiver operator characteristic curve analysis indicated that IVCCI under spontaneous breathing had the best area under the curve, with sensitivity of 1.0 and specificity of 0.98 for a cut-off of 0.22 to predict elevated CVP ≥ 10 mmHg. CONCLUSIONS: IVCCI seems to be a useful and accurate non-invasive index for estimation of elevated CVP in pediatric patients with cardiovascular disease.

[A retrospective study of advanced ovarian cancer in our ward].

OBJECTIVE: To evaluate the efficacy of primary treatment for ovarian cancer from overall survival and progression-free survival. PATIENTS AND METHODS: A total of 28 patients with epithelial ovarian cancer in stages III and IV who were primarily treated in our ward from 1993 were examined retrospectively. The Kaplan-Meier method and Harrington-Fleming test were carried out for the cumulative survival rate and analysis. RESULTS: There were significant differences in the progression-free survival rate depending on whether or not optimal debulking was possible through primary treatment (p = 0.0128) and whether the histological diagnosis was serous adenocarcinoma (p = 0.038). In the serous adenocarcinoma group, the periods of both overall survival and progression-free survival were longer in treatment with taxanes and platinum than by other regimens, but in the endometrioid adenocarcinoma group, the period of progression-free survival was very short. CONCLUSIONS: Optimal debulking through primary treatment is critical in advanced ovarian cancer. Therapy with taxanes and platinum is efficacious for serous adenocarcinoma. The chemo-sensitivity of endometrioid adenocarcinoma is high, but the chemotherapeutic effect is only temporary.