Quantitative evaluation of the pattern of shunt flow in the right ventricle and pulmonary artery of dogs with experimental ventricular septal defect.

Cineangiographic studies in patients with ventricular septal defect (VSD) have occasionally demonstrated that part of the blood across the defect is ejected immediately into the pulmonary artery (PA) passing through the outflow tract of the right ventricle (RV), but without being trapped in it. We attempted to make a quantitative evaluation of the flow of a partial shunt pathway (a direct VSD-PA pathway) that drains that part of the blood from the defect. Our method depended on a thermal dilution technique to obtain the ejection fraction of the RV and to observe a simultaneous pair of dilution curves at the roots of the aorta and PA after introduction of tracer into the left atrium. An analytical process was specially designed by incorporating a stable one-pass deconvolution technique. The method was applied to eight anesthetized dogs with acutely produced experimental VSD on the entrance of the outflow tract of the RV. The flow through the direct VSD-PA pathway was, in most cases, greater than 50 and up to 85% (mean of the eight, 57 +/- 5% SE) of the total left-to-right shunt flow. This would imply that less than 50%, and down to as little as 15%, of the total amount of shunt flow contributed to extra work of the RV in these cases. In addition, the impact on the pulmonary vasculature due to such a large amount of pulsatile flow through the direct VSD-PA pathway may accelerate the development of hypertrophy of the pulmonary vessel wall.

[Partial anomalous pulmonary venous connection to the high portion of the superior vena cava associated with ventricular septal defect repaired with williams method using right atrial flap: report of a case].

A 3-year-old boy suffered from the poor body weight gain under the diagnosis of partial anomalous pulmonary venous connection (PAPVC) to the high portion of the superior vena cava (SVC) associated with ventricular septal defect (VSD) and intact atrial septum (IAS). Preoperative cardiac catheterization revealed the pulmonary to systemic flow ratio 2.6 and mean pulmonary arterial pressure of 21 mmHg. He successfully underwent surgical repair with Williams method using right atrial (RA) flap out and VSD patch closure. Postoperative course was uneventful and echocardiography demonstrated no obstruction at the both routes of neo-SVC and neo-right upper pulmonary venous return.

[Aortic valve replacement for the patient with Osler-Rendu-Weber disease].

Osler Rendu-Weber (O-R W) disease (hereditary haemorrhagic telangiectasia) is characterized by an anormality of the peripheral blood vessels which often causes recurrent epistaxis. We presented a case of aortic valve replacement for the patient with O-R-W disease. A 75-year-old woman diagnosed as O-R-W disease with chest pain and dyspnea was admitted. Preoperative transthoratic echocardiography showed severe aortic valve stenosis (AS) with a pressure gradient (from left ventricle to ascending aorta) of 72.5 mmHg. Urgent aortic valve replacement (AVR) was performed after preoperative hormonal (estrogen-progesterone) therapy, that might be effective for bleeding from vascular ectasia. No hemorrhagic tendency was recognized and the postoperative course was uneventful.

[Surgical treatment for postinfarction ventricular septal perforation].

Between November 1985 and April 2003, surgical treatment for ventricular septal perforation (VSP) after acute myocardial infarction was performed in 16 patients. Patients were divided into 2 groups by method of operation. One group is infarct exclusion technique (n = 10). The other group is trans right ventricular (RV) approach (n = 6). No significant differences were observed between 2 groups in preoperative states. Operative death rate was high in both groups. Five patients (50%) were died in infarct exclusion group, 4 patients (67%) in trans RV group. Infarct exclusion technique needed longer extracorporeal circulation time (201 +/- 33 min) than trans RV approach (170 +/- 32 min). Although trans RV approach is attractive for its simplicity, 3 cases died of rupture of remained left ventricle infarction area. On the other hand, there were no mortality cases caused by left ventricle rupture in infarct exclusion technique group. As this result, we select infarct exclusion technique as a surgical correction for VSP.

[Surgical treatment for the patient with atrial septal defect and Andersen syndrome].

An 18-year-old girl with atrial septal defect and Andersen syndrome is reported. Andersen syndrome was described as a hereditary disease characterized by periodic paralysis, prolongation of the QT interval with ventricular arrhythmia and characteristic physical features including low set ear and micrognathia. We successfully performed cardiac operation for this rare associated malformation.

[Successful repair of supravalvular aortic stenosis using Steinberg 3 sinuses reconstruction; report of a case].

Steinberg method is a modification of Doty extended aortoplasty for supravalvular aortic stenosis (SAS). This modification entails placement of an additional patch in the left coronary sinus. A 3-year-old boy was diagnosed as SAS with aortic valvular stenosis. He was noticed a systolic murmur from 1 month after his birth. Echocardiography showed left ventricular hypertrophy, and pressure gradient of 80 mmHg was measured between the ascending aorta and the left ventricle. Cardiac catheterization revealed severe aortic stenosis at the sino-tubular (ST) junction. We adopted Steinberg 3 sinuses reconstruction. After this operation, there was no pressure gradient at ST junction although aortic valvular stenosis remained and mild aortic valve regurgitation newly developed. As this method can produce a symmetric aortic root, it may reduce aortic valve deformity especially on the left coronary cusp.

[Successful correction of incomplete endocardial cushion defects in an elderly patient].

We have experienced surgical correction of incomplete ECD in a 66-year-old female patient. This is the oldest case in Japanese literatures. Preoperative examination showed mild mitral regurgitation, interatrial shunt (L-R 52%) and moderate pulmonary hypertension (Pp/Ps 0.48). The correction consisted of valvuloplasty of mitral valve and patch closure of ostium primum. The postoperative course was uneventful. The postoperative catheterization showed improved cardiac function.

[Valvuloplasty for mitral regurgitation associated with acromegaly: report of a case].

A 67-year-old female with mitral regurgitation associated with acromegaly was admitted to our hospital. The cause of MR was torn chordae of posterior leaflet of the mitral valve. A prolapse part of the posterior leaflet was resected and sutured by McGoon's method. Annuloplasty was performed by Kay's method. Postoperative course was uneventful. She recovered well after the operation.

[The isolated congenital mitral regurgitation due to a partial defect of the anterior leaflet].

The isolated congenital mitral regurgitation (MR) is a rare anomaly. We have successfully treated a 7-year-old boy who had MR due to a partial defect of the anterior leaflet. Echocardiogram revealed moderate MR at the anterior leaflet as a result of its partial defect. Another MR was also detected at the coaptation zone of the 2 leaflets due to mild prolapse of the anterior leaflet. The mitral valve was approached via atrial septostomy and a partial defect (8 x 6 mm) was found near the postero-medial commissure of the anterior leaflet. Any chordae did not develop around the defect or subvalvular lesions. Other parts of the leaflet was seemed normal and any inflammatory or degenerative changes were not detected in the rest of the leaflets. We have done direct closure of the defect of the leaflet and also added annuloplasty at the posteromedial commissure by modified Kay method.

[Prostacyclin oral internal therapy for severe pulmonary hypertension after total correction of cor triatriatum with partial anomalous pulmonary venous return; report of a case].

A 37-day-old girl having cor triatriatum with partial anomalous pulmonary venous return was found on emergent admission. Echocardiography and angiography revealed severe pulmonary hypertension with anomalous pulmonary venous return to inominate vein. She still was in respiratory failure after emergent surgical repair because of severe pulmonary hypertension following pulmonary venous obstruction. Prostacyclin is the first Food and Drug Administration (FDA)-approved treatment for advanced primary pulmonary hypertension, which has antithrombotic properties related to its effect on platelets and is a potent vasodilator of both the systemic and pulmonary arteries, and has positive inotropic properties. We started prostacyclin oral internal therapy for her and she recovered from respiratory problems very quickly. Prostacyclin might be effective for pulmonary hypertension before and after surgical repair as primary pulmonary hypertension.

[A case report of aneurysm of the diverticulum of the ductus arteriosus].

A 59-year-old man who was admitted with hoarseness and diagnosed as aneurysm of the diverticulum of the ductus arteriosus was reported. In our case, enhanced computed tomography and aortography gave reliable informations. Operation was performed through a posterolateral thoracotomy under partial cardiopulmonary bypass. The aneurysm was successfully resected and defect of the aortic wall was covered with Dacron patch. Postoperative course was uneventful. To our knowledge, this is 12th surgically treated case in Japan.

[HANP infusion therapy for early infants with congestive heart failure after surgical repair].

We report about successful case of human atrial natriuretic peptide (HANP) infusion therapy for early infants who developed congestive heart failure after surgical repair of congenital heart diseases at Ehime Prefectural Central Hospital between January, 1998 through January, 2000. Age at operation ranged from 0 day to 38 days (mean 20 days), and body weight ranged from 1.6 kg to 3.5 kg (2.8 kg as mean). Following HANP infusion at incremental doses of 0.05 to 0.24 microgram/kg/min (0.16 microgram/kg/min as mean), urine output and hemodynamics dramatically improved. Urine output increased from 0.75 ml/kg/hr to 2.79 ml/kg/hr (p = 0.0001) and good urinary output was maintained (3.06 ml/kg/hr) even after discontinuation of the infusion. During the infusion, the heart rate decreased from 157 bpm to 150 bpm and the systemic systolic blood pressure increased from 68 mmHg to 78 mmHg. Central venous pressure decreased from 9.3 mmHg to 7.9 mmHg. There were no adverse effects by HANP infusion. These are successful cases of HANP infusion therapy as the initial treatment of post-operative pulmonary hypertension in this age group. Human atrial natriuretic peptide infusion therapy can be used safely and might be useful in some early infants with CHF, although more investigation is needed.

[The effect of milrinone administrated before weaning from cardiopulmonary bypass in adult cardiac surgery].

We investigated the effect of milrinone (phoshphodiesterase III inhibitor) on postoperative hemodynamics in adult eighteen cardiac surgical patients (mean LVEF = 63%). Milrinone was administrated just after the aortic declamping during CPB. Comparing with control group, systemic vascular resistance decreased significantly and sufficient inotropic effect was sustained. In order to keep proper blood pressure much more cathechoramine dose was need when milrinone dose increased during this study. This is supposed that milrinone affected strong vasodilating effect and caused relative hypovolemic condition to the patient who showed good cardiac function. Milrinone causes best efficacy to the patient of poor cardiac function when he showed deconpensation or relatively hypervolemic hemodynamic condition.

[Total anomalous of pulmonary venous connection in infants less than 3 months old].

We examined the surgical results of total anomalous pulmonary venous connection (TAPVC) retrospectively in 6 infants, who were less than 3 months old and underwent a total repair at Ehime Prefectural Central Hospital between May, 1993 through May, 1998, in terms of the pre, peri, and postoperative management, the site of connection, and the surgical procedures. Aged at operation ranged from 1 day to 86 days (mean 39 days), and body weight ranged from 2.4 kg to 5.5 kg (mean 3.4 kg). All 6 patients had echocardiographic diagnosis and cardiac catheterization but one. In operative procedure, cut back method was done in a patient of paracardiac type of Darling's classification and posterior approach was used in total correction for 4 supracardiac and 1 infracardiac type. There were 3 hospital deaths who had poor conditions before operation, but no late deaths. Surgical results of TAPVC might have been improved with advances in non-invasive diagnosis by echocardiography, and pre and perioperative management. And we should take care of these patients of TAPVC in long term period to make sure that they have no pulmonary venous obstruction.

[The present status and problems in artificial heart and lung apparatus and its clinical use].

The present status and problems in the usage of pump oxygenator were analysed through the answer obtained by questionaires which were sent to the 32 of major institution throughout our country. The total number of artificial heart and lung apparatus in use at 32 institutions were 5694, 5472 of roller pump and 222 of pulsatile pump, which showed predominantly in roller tyle. As for the artificial lung, 3151 were bubble oxygenator and 2172 were membrane type, membrane type appeared to be more popular. Only 4 institutions were satisfied with the use of present apparatus, rest of them were not satisfied and preclaimed certain demand of improvement of instrument, especially in 12 institution, compactrization of instruments were advised. The safety of the extracorporeal circulation with the use of present apparatus were accepted by 10 institution, however, complications such as hemolysis and organ failures like renal insufficiency were listed by 21 institutions. It appears to be concluded that much effort to improve the present pump oxygenator regarding to compactirization, safeness and automatic control of the machine are mandatory.

[Operative results of atrioventricular canal defect].

Partial Atrioventricular Canal Defect ( PAVC ) The operative results of this anomaly is generally acceptable. Between 1978 and 1982, 32 patients with PAVC underwent intracardiac repair. Two operative deaths occurred in 41 and 42 years old patients who were in NYHA class 4 preoperatively. Postoperative mitral insufficiency was either nil or mild except in one patient with mitral valve prolapse which was left unrepaired. Complete Atrioventricular Canal Defect ( CAVC ) Our operative procedures for the repair of CAVC are as follows, 1) all of accessory chordae are cut off and the bridging leaflet is incised into mitral and tricuspid components. 2) Ventricular septum, atrioventricular valve and atrial septum are constructed with our designed endocardial cushion prosthesis (Dacron prosthesis with pericardial brim of 3-4 mm width in each side). Between 1978 and 1982, 20 patients with CAVC containing 16 infants less than 2 years old underwent intracardiac repair using this prosthesis. Thirteen patients had no severe associated anomalies and only one death occurred in this group. Three patients were combined with tetralogy of Fallot (one died) and two patients with double outlet right ventricle (one died). The other two patients had either coarctation of the aorta (died) or interruption of the aortic arch (survived).