Tomohiko Ukai

Long-Term Outcome for Right Heart Function After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

OBJECTIVES The right heart function of the patients with pulmonary atresia and intact ventricular septum was assessed periodically during the process of staged biventricular repair, and the implications for its long-term outcome were analyzed. SUBJECTS AND METHODS During the period from 1971 to 1990, 21 neonates or infants with pulmonary atresia and intact ventricular septum had undergone initial palliative surgery. There were seven early postoperative deaths and one late death. Of the 13 survivors, 10 patients underwent subsequent biventricular repair and form the basis of this study. Their clinical records of roentgenography, electrocardiography, and catheterization studies at each staged period were reviewed retrospectively. RESULTS Arrhythmia occurred late in 2 patients, one of whom died by arrhythmia at 11 years after definitive surgical repair. Therefore the actuarial survival rate was 85.7% at 14 years. The catheterization study after the definitive biventricular repair revealed a significant fall in the right heart pressure (p = 0.0005) and significant improvement in the right ventricular ejection fraction (p = 0.0004). In angiocardiography, dilatation of the right atrium was noted in all patients and was more marked in those who developed arrhythmia in conjunction with rapid growth of the right ventricle. Moreover, the serial repeated electrocardiography disclosed progressive and significant prolongation of both PQ interval (p = 0.003) and QRS duration (p = 0.021). CONCLUSIONS Although biventricular repair for pulmonary atresia and intact ventricular septum proved to attain a satisfactory long-term result, it failed to resolve right heart dysfunction. Postoperative arrhythmia was prone to precipitate progressive dilatation of the right atrium.

Mitral regurgitation caused by ruptured chordae tendineae in Kawasaki disease

Kawasaki disease, a generalized vasculitis of unknown etiology, is becoming one of the leading causes of acquired heart disease in children. In the acute phase of illness, cardiac findings may reveal the involvement of the pericardium, myocardium, endocardium, and coronary arteries. Complications involving the coronary arteries are the most common causes of both early and late morbidity and mortality. I Valvular heart disease, such as primary mitral regurgitation, rarely occurs as a complication and may result in severe congestive heart failure. We report a case of mitral regurgitation caused by ruptured chordae tendineae in a patient with Kawasaki disease who underwent successful valve repair. Rupture of the chordae tendineae has not been previously reported as a cause of mitral regurgitation in Kawasaki disease. The patients Kawasaki disease was diagnosed at 3 months of age, when he had 5 days of fever, truncal rash, erythema of the palms and lips, bilateral conjunctival injection, and cervical lymphadenopathy. His serum C-reactive protein concentration was 9.1 mg/dl. He was treated 6 days after the onset of illness with high-dose aspirin and intravenous gamma globulin at a dosage of 400 mg/kg per day for 3 days. The patients clinical features improved rapidly, and his serum C-reactive protein concentration decreased to 0.7 mg/dl. His condition remained stable until day 24 of his illness, when a II/VI apical systolic murmur suddenly developed and his serum C-reactive protein concentration increased to 6.8 mg/dl. The patient had no fever at that time. Two-dimensional echocardiography and Doppler examination demonstrated mild mitral regurgitation without appreciable abnormalities of the coronary arteries. During a 3-day period, the patient came to have congestive heart failure as a result of mitral regurgitation. Intravenous gamma globulin was again administered to treat a recurrence of acute Kawasaki disease. Evaluation indicated rapid resolution of systemic inflammation, but severe heart failure resulting from significant mitral regurgitation persisted. Three months after the onset of illness, the patient was referred to our hospital for further evaluation. Cardiac catheterization confirmed the presence of grade 4/4 mitral regurgitation with associated pulmonary hypertension and a markedly dilatated left ventricle without evidence of hypokinesia or akinesia. Coronary angiography revealed

Protective effects of calpain inhibitor for prolonged hypothermic cardiac preservation

PURPOSE For successful organ transplantation, it is important to properly preserve the donor organ. This study was carried out to investigate tissue damage generated by the activation of calpain during prolonged hypothermic cardiac preservation using specific antibodies for mu- and m-calpain proenzymes, and to ensure the protective effect of calpain inhibitor 1 (N-acetyl-leucyl-leucyl-norleucinal). METHODS Excised rat hearts were divided into two groups: in Group I, the heart was arrested and immersed in University of Wisconsin solution with 20 microM of calpain inhibitor 1 (n = 28) and in Group N, the heart was arrested and immersed in University of Wisconsin solution without calpain inhibitor (n = 27). After a 12-hour preservation period at 4 degrees C, the hearts were reperfused on an isolated perfusion apparatus. Separation of the myocardial calpain isozymes was carried out by DEAE cellulose chromatography and both calpain proenzymes were detected by immunoblotting. RESULTS The cardiac function was more satisfactorily maintained in Group I in comparison with Group N. Remarkable leakage of creatine kinase, glutamic-oxaloacetic transaminase and lactate dehydrogenase was detected in Group N, while it was efficiently suppressed in Group I. During ischemia, mu-calpain proenzyme decreased in Group N (p < 0.01), but there was no significant change in m-calpain. However, during reperfusion, both mu- and m-calpains decreased more in Group N (p < 0.01). CONCLUSION Activation of calpain proenzymes and a decrease in cardiac function during preservation and reperfusion were demonstrated. The use of calpain inhibitor to protect against tissue damage was suggested as being useful for the prolonged preservation of the heart.

Ruptured Left Coronary Sinus of Valsalva Aneurysm Into the Left Ventricle

Ruptured aneurysm originating from the left coronary sinus toward the left ventricle (LV) is an extremely rare problem and the incidence was reported as 1.8% of all ruptured sinus Valsalva aneurysms [1]. This can cause severe aortic regurgitation, coronary insufficiency, and paroxysmal ventricular fibrillation [2]. A 59-year-old Japanese male presented with exertional dyspnea. Chest roentogenogram revealed bilateral pleural effusion and cardiomegaly. Two hours after his admission he required resuscitation because of sudden cardiopulmonary arrest. A two-dimensional echocardiogram demonstrated severe aortic regurgitation with compensated LV contractility. Initially he was treated with intensive medical care for congestive heart failure. Definitive diagnosis was confirmed on left-sided catheterization. The aortogram showed the “wind-sock” appearance of the aneurismal sac arising from the left coronary sinus extruding into the LV (arrow in Fig 1). The left coronary artery was intact (arrowhead in Fig 1) and no associated lesion, such as ventricular septal defect, was identified. Standard cardiopulmonary bypass was used during repair. The aneurysm was exposed through an oblique aortotomy. The aortic valvular ring at the left coronary sinus had detached completely from the aortic wall. The sac tightly adhered to the free wall of the LV and the bottom of the sac had perforated (asterisk in Fig 2). The left coronary cusp, valvular ring (arrowheads in Fig 2), and free wall of the aneurysmal sac were removed together. In order to obtain firm anchorage of a mechanical valve, mattress sutures with Teflon pledgets at the defect of valvular ring were directly placed on the aortoventricular junction where aneurismal wall adhering to the endocardium had turned into scar tissue. Histologic examination showed an accumulation of inflammatory cells (not only mononuclear cells but also neutrophiles) implying that a possible cause of aneurysmal formation was an infective endocarditis although any organisms could be identified from these specimens.

Infected pseudoaneurysm following a modified Blalock-Taussig shunt procedure

A 3-year-old boy with pulmonary atresia with ventricular septal defect, who had undergone placement of a modified Blalock-Taussig shunt, presented with a 1-week history of high fever. Computed tomography showed a pseudoaneurysm at the anastomosis between the right brachiocephalic artery and the graft. After intravenous antibiotic therapy, the pseudoaneurysm and infected graft were resected through a median sternotomy. This report describes successful management of a potentially fatal complication following placement of a modified Blalock-Taussig shunt.

Anatomical repair of aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery.

Aortopulmonary window (APW) with an anomalous origin of a coronary artery is extremely rare. We report surgical management of a four‐week‐old infant with the association of a distal type of APW and an anomalous origin of the right coronary artery (RCA) from the pulmonary artery. Complete anatomical correction comprising division of the great arteries and transferring the RCA as an autologous flap to the aortic defect was successfully performed. doi: 10.1111/jocs.12205 (J Card Surg 2013;28:767–769)

Pulmonary blood flow regulates plasma tissue plasminogen activator concentrations in patients with congenital heart defects

OBJECTIVE The wall shear stress generated by blood flow regulates the expression of fibrinolytic proteins by endothelial cells in vitro. In the present study, the effects of pulmonary blood flow on fibrinolytic activity were studied in patients with congenital heart defects and pulmonary hypertension. METHODS Twenty-seven patients who underwent cardiac operation because of congenital heart defects were divided into four groups according to the severity of pulmonary hypertension. Group I consisted of seven patients with normal pulmonary artery pressure, group II consisted of nine patients with pulmonary hypertension caused by increased pulmonary blood flow, group III consisted of six patients with pulmonary hypertension caused by increased pulmonary vascular resistance, and group IV consisted of five patients with tetralogy of Fallot. Plasma concentrations of tissue plasminogen activator, plasmin, and thrombin were assayed as the inhibitor-bound forms. RESULTS The preoperative concentration of tissue plasminogen activator was higher in group II than in all other groups (p = 0.0003). However, the postoperative concentration decreased only in patients in group II when compared with the preoperative value (p = 0.01). By Pearsons correlation analysis, pulmonary blood flow was found to correlate with the preoperative concentration of tissue plasminogen activator (95% confidence interval = 3.99 to 10.58, p = 0.0001). No definite conclusion was found for the relationship between tissue plasminogen activator and plasmin concentration. Further, the preoperative thrombin concentration was similar in all groups. CONCLUSIONS These findings suggest that pulmonary blood flow may regulate the plasma concentration of tissue plasminogen activator in patients with congenital heart defects.

Replacement of the Extracardiac Conduit for Infective Endocarditis After a Fontan Operation

We report the management of infective endocarditis after an extracardiac Fontan operation. Obstruction of the infected conduit and the associated pulmonary arterial embolism led to failure of the Fontan circulation. This was managed by completely removing both the infected conduit and emboli and replacing the conduit. doi: 10.1111/jocs.12266 (J Card Surg 2014;29:265–267)

Aortic coarctation repair in neonates with intracardiac defects: the importance of preservation of the lesser curvature of the aortic arch.

The aim of this study was to evaluate the mid‐term outcomes of a strategy for repair of coarctation of the aorta (CoA) and hypoplastic aortic arch (HAA) with a modified, extended end‐to‐end repair that preserves the lesser curvature of the aortic arch in neonates with intracardiac defects.

A review of extracorporeal membrane oxygenation in child after cardiac surgery: analyses of outcomes

Results Age and weight were 12 ± 17months and 6.4 ± 3.3kg, respectively. Twenty patients had single ventricle and 38 had biventricular physiology. The duration of ECMO was 8.4 ± 4.4 days. Fifty-two (84%) were successfully weaned off ECMO and 34 (55%) survived to hospital discharge. The weaning from ECMO was affected by peak serum lactate during ECMO (risk ratio = 1.02, 95% CI: 1.003-1.036, p = 0.0181) and the diagnosis of isomerism (46, 0.0004-0.41, 0.0085) by multivariate analysis. Indication for ECMO and surgical procedures were not significant predictors. Factors associate with failure of hospital discharge despite successful decannulation were as follows: weight (0.32, 0.14-0.62, 0.0001), ECMO duration (1.3, 1.02-1.72, 0.0352), the duration of the day between decannulation and the first day of negative water balance after ECMO (1.3, 1.13-1.54, 0.0001), the use of nitric oxide gas after ECMO (8.4, 1.81-46.66, 0.0068). Survivor vs. non-survivor to the hospital discharge was 7.1 ± 4.1 vs. 10.0 ± 4.2 (days) in ECMO duration (p = 0.0073) and 2.1 ± 1.5 vs. 8.3 ± 10.2 (days) in the negative water balance (p = 0.0004).