Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis / 日本心臓血管外科学会雑誌

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.

Leaflet Extension Aortic Valvuloplasty and Mitral Valve Replacement for Congenital Bicuspid Aortic Stenosis with Severe Mitral Regurgitation in a Child / 日本心臓血管外科学会雑誌

A baby girl with a low birth weight was given a diagnosis of congenital bicuspid aortic stenosis and mitral valve prolapse. At the age of 40 days, she underwent balloon aortic valvotomy, but significant aortic regurgitation appeared afterwards. Another surgical intervention became necessary by the age of 20 months (weight, 5.7 kg), because of intractable heart failure mostly caused by exacerbated mitral regurgitation. We performed a leaflet extension valvuloplasty for the small bicuspid aortic valve using an autologous pericardium treated by glutaraldehyde. The mitral valve was replaced with an ATS-16AP valve. Although her postoperative course was complicated with mitral paravalvular leakage and poor left ventricular function, she was discharged from hospital 6 months post operatevely. Leaflet extension valvuloplasty is a surgical option for infants with a small aortic annulus, but the procedure could be the only solution in cases when Konno or Ross techniques are not suitable.

Left Ventricular Free Wall Rupture Followed by Papillary Muscle Rupture Combined with Acute Myocardial Infarction / 日本心臓血管外科学会雑誌

We described a patient with free wall rupture followed by papillary muscle rupture due to acute myocardial infarction. A 69-year-old man was transferred complaining of transient unconsciousness. His clinical history, electrocardiogram, and chest CT showed myocardial infarction with free wall rupture indicated that several days had passed since the onset. Coronary angiography showed occlusion of the right coronary artery and severe stenosis of the left anterior descending artery. Since cardiac rupture was at inferior wall and hemorrhage wasn't active, repair of the rupture using fibrin glue and fibrin sheet and coronary artery bypass grafting to the left anterior descending artery was performed without cardiopulmonary bypass. On the 10th postoperative day, his arterial oxygen saturation suddenly deteriorated. Transesophageal echocardiography revealed papillary muscle rupture and severe mitral regurgitation. Emergency mitral valve replacement was performed. After two emergency operations, he gradually recovered and were discharged to home. In three months after discharge, he was admitted again due to congestive heart failure with left ventricular aneurysm at inferior wall and recovered in response of conservative treatment. Surgical experience of double rupture is rare. Based on this case, it may be necessary to perform reperfusion therapy toward even this case of recent myocardial infarction, to prevent papillary muscle rupture. It also may be better to use a patch on free wall rupture to prevent cardiac aneurysm.

Tricuspid Valve Plasty Using Autologous Pericardium for a Patient with Infectious Endocarditis / 日本心臓血管外科学会雑誌

A 27-year-old woman was given a diagnosis of infectious endocarditis with severe tricuspid regurgitation. Despite adequate antibiotics therapy, her general condition did not improve, and moreover multiple pulmonary abscesses were detected by computed tomography. Therefore surgery was indicated. Surgery consisted of removal of vegetation and tricuspid valve plasty with autologous pericardial patch augmentation of the anterior leaflet. Tricuspid valve plasty was carried out without prosthetic materials. Her postoperative course was uneventful with only mild tricuspid regurgitation. One year after surgery, neither recurrence of infection nor worsening of tricuspid regurgitation was noted. This method could be a useful technique for young patients with severe infection.

Surgical Removal of Left Ventricular Thrombi Combined with Acute Myocarditis / 日本心臓血管外科学会雑誌

A 47-year-old man had suffered from high grade fever and dyspnea for 10 days. He was transferred to our hospital in a condition of shock. Echocardiography showed severe diffuse hypokinesis of left ventricle (EF 21%), and multiple mobile thrombi in the left ventricle. Under a diagnosis of LV thrombi due to acute myocarditis, transatrial removal of LV thrombi was performed using video-assisted cardioscopy. He was weaned from cardiopulmonary bypass under IABP support. Postoperatively, he suffered from thromboembolism of the cerebral and right brachial artery. Thrombectomy of the right brachial artery and anticoagulation therapy was performed. IABP was removed on POD 3, and he no longer needed respiratory control on POD 4. Echocardiography on POD 6 showed marked improvement of the LV contraction (EF 52%). After rehabilitation, he was discharged on POD 23 on foot. Video-assisted cardioscopy allowed transatrial removal of LV thrombi, and preserved left ventricular function by avoiding ventriculotomy. Perioperative thromboembolism must be taken care of for a patient with multiple LV thrombi.

Surgical Correction for Congenital Valvular and Supravalvular Aortic Stenosis Associated with Coronary Ostial Stenosis in a Child / 日本心臓血管外科学会雑誌

Supravalvular aortic stenosis is a rare obstructive lesion of the left ventricular outflow tract localized at the level of sinotubular junction. It has been recognized that supravalvular stenosis may occur as a part of Williams syndrome and is sometimes complicated by obstruction of the left main coronary artery. We successfully performed single patch augmentation for supravalvular aortic stenosis and left coronary ostial stenosis with concomitant aortic valvotomy in a child without Williams syndrome. The patient had been followed as congenital bicuspid aortic valvular and supravalvular stenosis. At the age of 3 years, cardiac catheterization revealed an increased pressure gradient of 90mmHg at the left ventricular outflow and newly developed ostial stenosis of the left coronary artery. An oblique incision on the ascending aorta was made above the sinotubular junction and extended leftward onto the left main coronary artery, and this incision opened the fibrous ridge at the left coronary artery. After commissurotomy for the bicuspid valve, both the supravalvular and ostial stenosis were augmented with a single autologous pericardial patch treated by glutaraldehyde. The pressure gradient was significantly reduced and the ischemic left ventricular dysfunction was eliminated.

Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy in a Child / 日本心臓血管外科学会雑誌

A 6-year-old boy who had been found to have hypertrophic obstructive cardiomyopathy presented with severely limited symptoms of heart failure due to progressive left ventricular outflow obstruction. Cardiac catheterization revealed the peak systolic pressure gradient of 87mmHg at left ventricular outflow, and systolic anterior motion of the anterior mitral leaflet with concomitant mitral regurgitation was observed by echocardiography. Transaortic septal myectomy was performed using transesophageal echocardiography guidance before, during and after surgery. Although the patient needed permanent pacemaker implantation for postoperative complete heart block, the procedure reduced the left ventricular outflow obstruction and relieved his symptoms.

Damus-Kaye-Stansel Anastomosis for Rapid Progression of Subaortic Stenosis after Pulmonary Artery Banding in a Single Ventricle Infant with Aortic Arch Hypoplasia / 日本心臓血管外科学会雑誌

A cyanotic baby boy was given a diagnosis of single right ventricle, double outlet right ventricle, hypoplastic aortic arch, mitral atresia, atrial septal defect and pulmonary-ductus-descending aorta trunk. On day 4, extended aortic arch anastomosis and pulmonary artery banding were undertaken. At age 70 days, severe cyanosis and respiratory distress appeared and advanced rapidly. Angiography revealed critical subaortic stenosis and pulmonary hypertension, and the patient required urgent Damus-Kaye-Stansel anastomosis with concomitant right modified Blalock-Taussig shunt. Patients with single ventricle and hypoplastic aortic arch are a high-risk subgroup of progressive subaortic stenosis after initial pulmonary artery banding, and therefore need careful observation and may require early relief of subaortic stenosis.