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
A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.
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
A 67-year-old man with dilated cardiomyopathy was admitted to our hospital for treatment of cardiac failure. After using heparin because cerebral infarction developed during hospitalization, in acknowledgment of thrombocytopenia, we reach the diagnosis of HIT. We judged surgery to be necessary because heart failure had difficulty with catecholamine secession and the left ventricular dilation progressed rapidly, and performed left ventriculoplasty, mitral valve plasty. There were no complications such as the thrombosis during cardiopulmonary bypass, and the postoperative course was good without leading to re-thoracotomy due to bleeding. He passes without a heart failure symptom by the follow of one year 6 months after surgery at home.
ABSTRACT
<p>We report 4 cases of aortic graft replacement for Kommerell diverticulum (KD) and the aberrant subclavian artery (ASA). In two patients who had a right-sided aortic arch, KD and the left ASA, we performed descending aorta replacement and <i>in-situ </i>reconstruction of the left ASA via a right lateral thoracotomy. Third patient had a left-sided aortic arch, KD and the right ASA, in whom we performed descending aorta graft replacement via a left lateral thoracotomy with ostial closure of the right ASA. Fourth patient had a left-sided aortic arch, KD and the right ASA, and complicated by acute type A aortic dissection. We performed a total arch repair with frozen elephant trunk procedure via a median sternotomy. All 4 patients survived operations and discharged from the hospital with symptom relief. The choice of approach, a thoracotomy or a median sternotomy, should be based on patient-specific anatomy and extent of graft replacement.</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.