[Intracardiac repair of tetralogy of Fallot with an anomalous coronary artery crossing the right ventricular outflow tract].

Between may 1993 and march 2001, 2 patients with tetralogy of Fallot and an anomalous coronary artery crossing the right ventricular outflow tract underwent intracardiac repairs. The anomalous coronary arteries included the left anterior descending from the right coronary artery (case 1), and the right coronary artery from the left coronary artery (case 2). In case 1, we turned down a flap of anterior wall of the main pulmonary artery, sutured it to the edge of the right ventriculotomy and placed a bicusped patch to the anterior aspect. In case 2, we underwent transpulmonary-transatrial repair and placed a transannular patch along by the left coronary artery. Right ventricular outflow tract reconstruction was successful in 2 cases.

Absent aortic valve: successful palliation in the neonate.

A successful palliation of a neonate with absent aortic valve in hypoplastic left heart syndrome is reported.

[Cases of multiple ventricular septal defects].

We had 4 cases with multiple ventricular septal defects (VSDs) in complexed congenital heart disease. One of four had two separate VSDs detected by two dimensional echocardiography before operation. Second of four had additional infundibular muscular VSD which was detected by echocardiography in the intensive care unit (ICU) after patch closure of a perimembranous VSD. The third case had two additional VSDs of inlet muscular and subaortic septum detected by transesophageal and direct echocardiography during reoperation, beside a subpulmonary VSD which was originally diagnosed before Jatene operation for double outlet right ventricle. The fourth case had multiple trabecular muscular VSDs diagnosed by postoperative angiography soon after Rastelli operation. Since these additional multiple VSDs compromise the postoperative hemodynamics if those are unrecognized, it is indispensable to detect all VSDs before operation, using transthoracic and transesophageal echocardiography.

[A case of severe cytomegalovirus infection after the repair of coarctation of aorta].

We report a 2-month-old boy without any immuno-compromised diseases, who suffered from the severe cytomegalovirus (CMV) infection after the subclavian flap aortoplasty and pulmonary artery banding for coarctation complex. He underwent the operation at 2 months old and received 2 units of irradiated packed red blood cells before and after the surgery. His postoperative course was uneventful but the interstitial pneumonitis, until he developed watery diarrhea 10 days after the surgery following hepatitis with the marked hepatomegaly 3 weeks after. Since CMV infection was confirmed as the cause of the pneumonitis, enterocolitis and hepatitis, he was initially treated by gamma-globulin with the high CMV titer at a dose of 200 mg/kg/day for 2 days and ganciclovir at a dose of 10 mg/kg/day for 14 days. Because of the persistent CMV infection, he needed two more treatments of ganciclovir at the same dosage and gamma-globulin once a week for 2 months. He finally recovered from severe CMV infection 5 months after the above treatments. In conclusion, the severe CMV infection can occur by blood transfusion even in the surgical case with normal immune system. If one finds pneumonitis, hepatitis or enterocolitis after any type of surgery with history of blood transfusion, CMV infection should be suspected as the cause of these diseases.

[Two cases of complete atrioventricular canal defect whose pulmonary vascular resistance were over 10 wood unit.m2 before 6 months of age].

We report two cases with complete form of atrioventricular canal defect (CAVCD) accompanied by Down's syndrome whose pulmonary vascular resistance (Rp) were more than 10 Wood unit.m2 at the age of less than 6 months. One child was 3-month-old boy whose Rp was 12 Wood unit.m2. The open lung biopsy at 3 months old showed histopathological change of Heath-Edwards grade I. He underwent intracardiac repair at the age of 4 months. He is doing well at 30 months of postoperative period. Another child was 5-month-old girl whose Rp was 15.5 Wood unit.m2. Histopathological change of lung at 5 months old demonstrated Heath-Edwards grade III. She underwent intracardiac repair at the age of 7 months, 2 months after lung biopsy. However, she died of oversystemic pulmonary hypertension and low output syndrome 7 days after surgery. The postmortem examination revealed that pulmonary vascular obstructive disease progressed during 2 months interval between the lung biopsy and the operation. In conclusion, cardiac catheterization with estimation of Rp should be performed in the cases of CAVCD, especially in those with Down's syndrome, in early infancy. If Rp is more than 10 Wood unit.m2 and lung biopsy indicates the surgical indication, surgical intervention should be done as soon as possible since the pulmonary vascular obstructive disease may progress in a short period.

[Anomalous origin of the right pulmonary artery from the ascending aorta: a case report].

We report a successful case of anomalous origin of the right pulmonary artery from the ascending aorta. A 34 days old infant, who had suffered from cardiogenic shock, underwent a direct anastomosis of the right pulmonary artery to the main pulmonary artery behind the ascending aorta under cardiopulmonary bypass. Postoperative catheterization showed no significant stenosis over the anastomosis and normalized pulmonary artery pressure. We conclude that early surgical correction is necessary in the treatment of this life threatening disease.

[Two cases of gross E-type tracheo-esophageal fistulae associated with interruption of the aortic arch and coarctation of the aorta in early infancy].

Two cases of Gross E-type tracheoesophageal fistulae associated with interruption of the aortic arch in one and coarctation of the aorta in the other in early infancy were treated radically at Nagano Children's Hospital during the preceding 4 months until January 1994. The tracheoesophageal fistulae were noticed after the first stage surgery for the aortic arch anomalies, because of the remarkable abdominal distension under the intubated and ventilated condition of general anesthesia. Both the bronchial and esophageal optical fiber examinations were performed which proved useful to detect the fistulae. In each case, the transcervical division of the fistula was performed on an emergency basis. The external diameter of the fistula was 4 and 5 mm respectively. The fistulae dilated synchronously with ventilation. The early detection and surgical correction of the tracheoesophageal fistula can prevent serious complications such as DIC as seen in the first case probably caused by respiratory infection associated with the prolonged mechanical ventilation. Successful intracardiac repair were performed in both cases on the 25th and 7th day following the correction of the fistulae respectively.

Combined assessment with myocardial contractility and an index related to ventricular afterload in patients with isolated severe mitral regurgitation undergoing valve replacement. A retrospective study.

The afterload to the left ventricle increases following valve replacement in chronic mitral regurgitation (MR), yet there has been no study taking into account left ventricular (LV) afterload. In 43 patients with isolated chronic MR undergoing mitral valve replacement (MVR), we calculated LV wall stress using preoperative LV end-diastolic dimensions and diastolic aortic pressure (predictive LV wall stress = pWS). This value would represent LV wall stress at the instant of aortic valve opening immediately after MVR, assuming that LV end-diastolic dimensions and the aortic pressure did not change after operation. We also calculated end-systolic wall stress/volume index (ESS/ESVI) ratio, an index of myocardial function. None of 15 patients with the ESS/ESVI ratio > 1.6 had postoperative mortality or morbidity, whereas among the 26 patients with the ESS/ESVI ratio < 1.6, 12 patients had mortality (3 patients) and/or morbidity requiring intraaortic balloon pumping or prolonged catecholamine support. Three patients who died had pWS > 200 kdynes/cm2. Including these 3, all 5 patients whose pWS > 200 had mortality or severe morbidity immediately after surgery. In contrast, among the 38 patients with lower pWS, none died and only 7 patients (18%) had morbidity. Thus, patients who had a low contractility index and a high predictive afterload value had high mortality and morbidity. We conclude that the prediction of postoperative mortality and morbidity is improved by combined assessment of myocardial function and the hypothetical postoperative LV afterload.

Prediction of surgical result of valve replacement for chronic isolated mitral regurgitation--the significance of preoperative estimation of postoperative left ventricular afterload.

Left ventricular (LV) afterload increases after mitral valve replacement, thus it would be useful to estimate postoperative LV afterload before surgery. We tested the usefulness of an index, wall stress, obtained from preoperative end-diastolic LV dimensions and diastolic blood pressure which would represent LV afterload after surgery. The data were compared with surgical mortality and morbidity. The wall stress ranged from 98 to 220 kdynes/cm2 and was 202 or higher in 3 patients who died. Five patients had wall stress above 200 kdynes/cm2. Among these, intra-aortic balloon pumping (IABP) was used in 4, and 3 died. Prolonged catecholamine support for greater than 10 days was given to all of the 4 patients, including two who died 14 and 23 days after surgery. Among 38 patients who had wall stress less than 200 kdynes/cm2, none died, IABP was performed in 3 patients, and prolonged catecholamine infusion was required in 5 patients. The incidence of mortality and morbidity were significantly higher in the high stress than in the low stress group (Chi-square analysis). Left ventricular end-diastolic index was larger in the high stress than in the low stress group (p less than 0.05). The mass/end-diastolic volume ratio was smaller in the high stress group than in the low stress group (P less than 0.05). In conclusion, this new index, predictive wall stress, is useful in selecting patients who would have high mortality and morbidity.

Effect of acidosis on intracellular pH and calcium concentration in the newborn and adult rabbit myocardium.

This study investigated developmental changes in the effect of acidosis on intracellular pH (pHi) and [Ca]i in the isolated heart and isolated myocyte preparations. The whole heart or myocytes of newborn (5-7 days old) and adult rabbits were loaded with the fluorescent pH indicator 2',7'-bis(2-carboxyethyl)-5(6)-carboxyfluorescein (BCECF) or calcium indicator fura-2. Left ventricular pressure in the isolated heart preparation and the magnitude of cell contraction in the single-cell preparation were monitored. The heart and single cell were illuminated with excitation lights (340 and 380 nm, respectively, for fura-2 and 438 and 490 nm for BCECF). The intensity of fluorescence from the ventricular surface or from the cell was detected. [Ca]i was estimated from the following ratio: fluorescence at 505 nm during excitation at 340 nm/fluorescence at 505 nm during excitation at 380 nm. pHi was estimated from the following ratio: fluorescence at 530 nm during excitation at 490 nm/fluorescence at 530 nm during excitation at 438 nm. In the newborn, depression of contractile function during respiratory acidosis or metabolic acidosis was less than in the adult. Diastolic and systolic [Ca]i increased during respiratory acidosis in both the newborn and adult, and the net changes in [Ca]i were similar in the two age groups. During respiratory or metabolic acidosis, pHi decreased, but the decrease in the newborn was significantly less than in the adult. These data suggest that the greater resistance of the newborn myocardium to acidosis is due to the smaller change of pHi in this age group and not due to the difference in [Ca]i alteration.