ABSTRACT
Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.
ABSTRACT
We sometimes encounter the case that we have to make an anastomosis between a prosthetic graft and an autologous vein graft in revascularization of a lower extremity. However, it is said that the intimal hyperplasia in the anastomosis site of a prosthetic graft and autologous vein graft has a tendency to become severe in the long term postoperatively. We herein report a case in which a vein cuff (St. Mary's boot) technique was very useful to prevent recurrent stenosis due to intimal hyperplasia. No recurrence of stenosis in repair lesion has been detected for 7.5 years after operation.
ABSTRACT
We present a successful case of a patient with multiple metal allergy—cobalt, nickel, chromium, and zinc—who had a past history of systemic metal dermatitis. He was a 58-year-old man who complained of exertional chest discomfort. After admission, he had a fever and his blood culture was positive with methicillin-sensitive Staphylococcus aureus. Three days later, multiple micro cerebral infraction was detected in magnetic resonance imaging. After an improvement of inflammatory reaction, he was transferred to our facility for cardiac examination. Moderate mitral regurgitation due to valve perforation and multiple coronary vessel stenosis were detected. Mitral valve replacement and coronary artery bypass grafting were planned to perform. We chose surgical materials based on a preoperative epicutaneous (patch) test and his clinical course was uneventful without any allergic reaction. Metal contact allergy is an important issue in cardiovascular surgery. A collaboration with dermatologists is essential for the preparation of surgical materials.
ABSTRACT
When mitral valve dysfunction occurs in infants and mitral valve repair is difficult, mitral valve replacement (MVR) is required. However, commercially available prosthetic heart valves can be too large to implant in infants with a small annulus. In these children, the technique of supra-annular MVR is useful. Here we report two cases of supra-annular MVR, which were performed using an expanded polytetrafluoroethylene (ePTFE) graft as a skirt for a prosthetic valve. This method has been previously reported by Sung et al. The first case was a 16-month-old, 6.7-kg male infant who suffered from Shone' syndrome, mitral stenosis (MS) with a parachute mitral valve, coarctation of the aorta (CoA), and ventricular septal defect (VSD). MS progressed after CoA repair and VSD closure and a supra-annular MVR was performed. The second case was a 5-month-old, 4.9-kg female infant who suffered from polysplenia, intermediate atrioventricular septal defect (AVSD), and severe left atrioventricular valve regurgitation. AVSD repair was performed at the age of 3 months. However, valve stenosis and regurgitation gradually progressed postoperatively and consequently, a supra-annular MVR was performed. Postoperative prosthetic valve function was good in both cases. We believe that this method of performing supra-annular MVR is useful for infants with a small annulus.
ABSTRACT
Due to the increasing number of patients with repaired tetralogy of Fallot (TOF), the amount of subsequent sequelae has also increased along with the need for further surgical interventions. This case report described a patient who underwent multiple reoperations for late sequelae over a period of 48 years after repair of TOF. The patient was a 58-year-old man who underwent a reparative operation for TOF at the age of 9 years. At the age of 30, he underwent patch closure of residual VSD and reconstruction of right ventricular outflow tract (RVOT) with a mono-cusped trans-annular patch combined with aortic valve replacement. At the age of 47, he underwent aortic root replacement for aneurysmal dilatation of the Valsalva sinus, and he was implanted with a pacemaker for bradycardia atrial fibrillation at the age of 51. By the age of 58, he required pulmonary valve replacement and tricuspid valve annuloplasty for right ventricular dysfunction resulting from regurgitation of the pulmonary and tricuspid valves as 4th midline sternotomy surgery. His status improved to NYHA functional class III to II after the most recent procedures, although he still needed strict medical control. A proactive approach during long-term follow-up after TOF repair is important for timely and appropriate surgical intervention in the event of late sequelae.
ABSTRACT
Gerbode defect is a communication between the left ventricle and right atrium. It is usually congenital rather than acquired, but can occur as a complication of endocarditis, myocardial infarction, trauma, or cardiac surgery. We report a case of surgical repair of acquired Gerbode defect resulting from infective endocarditis. A 69-year-old woman with aortic regurgitation due to infective endocarditis was referred to our hospital with a diagnosis of congestive heart failure. She was hospitalized and underwent medical treatment (intensive antibiotic therapy). Preoperative transthoracic and transesophageal echocardiography were performed and revealed a mobile mass (vegetation) on the aortic valve. The patient also had severe aortic regurgitation and a communication between the left ventricle and right atrium. The communication was visualized in the atrioventricular membranous septum. Due to the mobility of the mass (vegetation) and uncontrollable congestive heart failure caused by severe aortic regurgitation, surgical treatment was advised. Surgery was performed through a median sternotomy with the patient on cardiopulmonary bypass. After aortic cross-clamping, the vegetation was approached through a horizontal incision in the ascending aorta and a right atriotomy. The communication site from the left view was below the commissure between the right coronary and non-coronary cusps ; from the right view, it was just above the tricuspid annulus of the septal leaflet. The defect was closed with two 0.4-mm thick Gore-Tex cardiovascular patches : one was placed on the LV side and the other on the RA side. The aortic valve was replaced with a bioprosthetic valve (SJM Epic 21 mm). The operation was finished and her clinical course was almost uneventful.
ABSTRACT
<p>Calcified amorphous tumor (CAT) is a non-neoplastic cardiac mass composed of nodules of calcium with a background of amorphous fibrous material and was first described in 1997. This report describes a 61-year-old man, who had been on hemodialysis for 10 years and was referred to our hospital with a diagnosis of acute myocardial infarction. He had percutaneous coronary intervention (PCI) for stenosis of the left anterior descending artery (LAD). He was hospitalized and under medical treatment. A follow-up echocardiogram was performed and revealed a normal ejection fraction of 0.60. Moderate mitral annular calcification with mild-to-moderate mitral stenosis was seen. An ultrasound-mobile mass was visualized in the left ventricular outflow tract (LVOT). There was no hemodynamic evidence of LVOT obstruction on Doppler echocardiography. Transesophageal echocardiography showed a mobile mass attached to the LVOT in the mitral valve annulus that extended almost to the membranous septum. Due to the mobility of the mass and potential for embolism, surgical removal was advised. Concomitant procedures (coronary artery bypass grafting (CABG) and a full maze operation) were proposed because he still had symptoms of chest pain with myocardial ischemia and palpitations due to chronic atrial fibrillation. Surgery was performed through a median sternotomy on cardiopulmonary bypass. After aortic cross-clamping, the mass was approached through a horizontal incision in the ascending aorta. The white tumor was resected easily from the membranous septum. The operation was finished after CABG and a full maze procedure, and his clinical course was uneventful. Histological examination showed that the tumor contained many calcified nodules and fibrino-sanguineous deposits ; these findings are compatible with CAT.</p>
ABSTRACT
Type II endoleak after stent graft for abdominal aortic aneurysm generally permits a wait-and-see approach, however, additional second therapy is necessary if the aneurysm increases. A 78-year-old woman had undergone endovascular treatment with a stent-graft for abdominal aortic aneurysm. Computed tomography (CT) 3 months after the operation showed type II endoleak, and enlargement of the aneurysm was noted at 1 year. She underwent transarterial embolization for third and fourth lumbar arteries, but endoleak from the fourth lumbar artery remained, and the aneurysm was further enlarged. Surgical conversion was performed 2 years after the first operation. We applied a ligature to the forth lumbar artery by a laparotomy. Postoperative CT revealed disappearance of the endoleak. As endovascular treatment for abdominal aortic aneurysm is increasing and the number of cases necessitating additional therapy appears to be rising, we need to develop processes and strategies for coping with endoleaks.
ABSTRACT
A 68-year-old woman with a diagnosis of atrial septal defect (ASD) presented with dyspnea. Chest radiography demonstrated cardiomegaly and infiltration in both lungs, suggestive of cardiac decompensation due to ASD. Detailed evaluation with transthoracic echocardiography revealed a mobile tumor on the aortic valve. Intraoperatively, tumors were identified on all aortic cusps. Preservation of the aortic valve was difficult. We therefore performed aortic valve replacement and patch closure of the ASD. The existing literature suggests that mobile papillary fibroelastoma should be excised irrespective of size, to prevent the risk of embolism. Excision of the tumor alone is usually sufficient. However, the present case showed clustered tumors on the aortic valve, so preservation of the cusps could not be achieved in this case.
ABSTRACT
A sinus of Valsalva aneurysm is a rare cardiac disorder, and reports of it with an anomalous origin of the coronary artery are scarce. A 35-year-old male was admitted to our department with fatigue and cough. Multi-detector-row computer tomography (MDCT) revealed an isolated extracardiac right sinus of Valsalva aneurysm with an anomalous origin of the left circumflex artery (LCX) and total occlusion of the right coronary artery (RCA). Its diameter was about 70 mm. We performed a partial aortic root remodeling procedure with a trimmed J-graft because he had neither aortic regurgitation (AR) nor annuloaortic ectasia (AAE). Concomitantly, coronary artery bypass grafting to the RCA (Seg. 3) using a saphenous vein, and reconstruction of the LCX by Piehler's technique using a saphenous vein were added. The patient's postoperative course was uneventful, and he was discharged on the 28th postoperative day. Postoperative MDCT revealed that the aneurysm of the right sinus of Valsalva was not enhanced, and the RCA and LCX were patent. This procedure preserved the patient's own normal aortic valve and sinus of Valsalva and enables him to have more physiologically normal hemodynamics than aortic root reconstruction using a composite graft, e.g. Bentall procedure, Cabrol procedure, although the potential progression of the AR requires careful follow-up.