Case Of Successful Surgical Repair Research Articles

Surgical correction of a ventricular septal defect in a child with spinal muscular atrophy type 2 treated with nusinersen sodium: a case report

IntroductionSpinal muscular atrophy (SMA) is a severe, inherited neuromuscular disorder characterized by progressive muscle weakness and atrophy. Cardiac pathology co-existence is reported more frequently in the severely affected patient groups. Structural heart anomalies, mainly septal, and outflow tract defects are commonly observed pathologies.Case presentationWe herein report the case of a 23 days-old female patient with the diagnosis of spinal muscular atrophy type 2 complicated with structural heart defects. Successful pulmonary banding, and at the age of 17 months, subsequent surgical atrial and ventricular septal defect closure were performed on our patient who was under treatment of Nusinersen Sodium. Post-operative recovery was uncomplicated. Cardiac assessments were normal, and the patient was neurologically improving in her recent follow-up.ConclusionIn the literature, there are no reported cases of successful surgical repair of heart defects in spinal muscular atrophy patients. These patients can be perceived as risky surgical candidates with suboptimal postoperative recovery given the unfavorable disease prognosis of SMA in untreated patients. We report our promising experience with a SMA type 2 patient undergoing a disease-modifying medical treatment. The SMA patients under treatment may be potential candidates for successful surgical cardiac correction given their overall improved prognosis.

BILATERAL CALCIFIED URETERAL STRICURE REQUIRING URINARY RECONSTRUCTION IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGITIS: A CASE REPORT

Calcified ureteral obstruction is a rare complication of vasculitis. Only a few cases of eosinophilic granulomatosis with polyangiitis (EGPA) have been published. We herein report a case of successful surgical repair of bilateral ureteral strictures in a patient with EGPA. A retrograde pyelogram revealed complete bilateral ureteral obstruction in the patient. Bilateral nephrostomies were placed because we were unable to introduce a double-J stent. The vasculitis and eosinophilia both responded to immunosuppressive therapy, but ureteral obstruction persisted. Surgical reconstruction was elected in order to eliminate the need for permanent external drainage. A combined nephrostogram/retrograde ureterogram revealed a 10 cm right ureteral gap and a 3 cm left ureteral gap. The left ureter was amenable to end-to- end anastomosis, pyeloureteroplasty, but repair of the right ureter required interposition of intestinal tissue. Right ureteral reconstruction was performed by combining the Yang-Monti ileal ureteral substitution and appendiceal interposition with a psoas hitch. The isolated appendix was interposed in an antiperistaltic fashion between the Yang-Monti tube and bladder. His kidney function has remained normal, and he is without vasculitic symptoms.

Hammock mitral valve: A rare case report

Congenital mitral stenosis is a relatively rare disorder comprising 0.2% of all congenital heart defects. Hammock mitral valve producing severe mitral stenosis is a rare variant of congenital mitral stenosis. We report a 2-year-old boy who had hammock mitral valve producing severe mitral stenosis with severe pulmonary artery hypertension. He underwent successful surgical repair. Post-surgery, the mitral valve opening was adequate without residual stenosis or regurgitation. Pulmonary artery pressure had normalized. Follow-up data showed he had significant clinical and echocardiography improvement. This is the first reported case of successful surgical repair done for hammock mitral valve from our institute.

症例 特有顔貌を伴った Discrete 型大動脈弁下狭窄症の1手術治験例

症例 特有顔貌を伴った Discrete 型大動脈弁下狭窄症の1手術治験例

A case of successful surgical repair for solid segment type pyloric atresia using a novel gastroduodenostomy procedure

We present a case of successful surgical repair for solid segment type pyloric atresia using a novel gastroduodenal procedure.

Anomalous Left Coronary Artery from the Pulmonary Arteryin an Adult

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that usually presents before the age of 1 year. Several surgical options exist for the correction of ALCAPA; however, debate continues regarding the optimal repair technique in adult populations. We report the case of successful surgical repair of ALCAPA with a direct aortic implantation technique in a 44-year-old mother of 4 children.

Infective endocarditis complicated with left atrioventricular fistula in a 20-year-old patient. A case study

Intracardiac fistulas are rare complications of infective endocarditis. We report an unusual case of successful surgical repair of intracardiac fistula between the left ventricle and the left atrium in the course of infective endocarditis in a 20-year-old patient. According to this we conclude that timely diagnosis, proper antibiotic treatment, and early surgical intervention should improve the outcomes of infective endocarditis complications.

The window ductus: Circulatory arrest as an option for surgical repair

The window ductus represents a very rare anatomical anomaly. Only a few cases of successful surgical repair have been reported. We present a 12 year old boy in whom window ductus was diagnosed and successfully operated. The operation included closure of the large (2.5 cm×0.5 cm) connection between the main pulmonary artery and the aortic arch using cardiopulmonary bypass and hypothermia with circulatory arrest.

Type IV Laryngotracheoesophageal Cleft Associated with Microgastria and Left Lung Hypoplasia: Report of a Long-term Survivor

Laryngotracheoesophageal clefts (LTEC) are extremely rare congenital anomalies associated with a high attendant morbidity and mortality. LTEC are characterized by a midline communication between the esophagus and trachea extending from the larynx over a variable distance down to the bifurcation. The common proximal aerodigestive channel is a result of an incomplete separation of the primitive foregut into trachea and esophagus during fetal development. Clinical severity strongly depends on the length of the cleft. Many classifications have been proposed in order to distinguish the various clefts. Pettersson's anatomy-based classification divided laryngotracheoesophageal clefts into three groups according to their length. Later the classification was modified by Armitage [1] and Evans [9]. A clinically more relevant classification was offered by Benjamin and Inglis [10]: type I involves the supraglottic larynx, type II extends beyond the level of the vocal cords, type III is a total cricoid cleft with or without extension to cervical trachea, type IV is characterized by a complete extension of the cleft to the level of the carina with a common esophageal and tracheal lumen. Minor clefts account for the majority and only 3 % of all clefts are type IV. While type I may be repaired endoscopically or even treated conservatively, type II–IV clefts are fatal conditions and require surgical repair [3], [7], [21]. A review of the literature revealed a few reported cases of successful surgical repair of type IV LTEC but only a handful of long-term survivors [4], [13], [15], [27].

Berry Syndrome Two Cases of Successful Surgical Repair

Berry syndrome is a very rare congenital cardiovascular anomaly that consists of a distal aortopulmonary window, aortic origin of the right pulmonary artery, an intact ventricular septum, a patent ductus arteriosus, and an interrupted aortic arch. Two cases of Berry syndrome are presented. A one-stage surgical correction for this complex anomaly was successfully performed, and a 1-year follow-up demonstrated favorable outcomes.

Infective Endocarditis and Pseudoaneurysm: Fatal Complications of Mitral Annular Calcification

We present a case of successful surgical repair for infected mitral valve and left ventricular pseudoaneurysm, which can be fatal complications of mitral annular calcification.

Papillary Muscle Rupture After Blunt Chest Trauma

We report a case of anterolateral papillary muscle rupture in a 22-year-old man who had blunt chest trauma caused by a car accident. Transesophageal echocardiography revealed severe mitral regurgitation caused by the rupture. He successfully underwent emergency mitral valve replacement and was discharged 9 days after the surgical correction.

Popliteal artery occlusion associated with dislocation of the knee-joint. Report of a case with successful surgical repair

Abstract The ninth case of successful surgical repair of popliteal artery injury associated with dislocation of the knee-joint is reported. Emphasis is placed on the limited time interval between injury and surgical intervention in order to achieve restoration of the circulation.

Surgical correction of a patient with discordant atrioventricular and concordant ventriculo-arterial connections

AbstractThe combination of discordant atrioventricular and concordant ventriculoarterial connections is an extremely rare entity, with only 8 cases of successful surgical repair being previously reported. Although from the clinical standpoint this anomaly is similar to complete transposition, this unusual combination is clearly suited by its morpho-physiological features to an atrial rather than an arterial switch procedure. A 17-year-old boy with this malformation, associated also with severe pulmonary hypertension, underwent a palliative Senning procedure, leaving the ventricular septal defect open and banding the pulmonary trunk. Postoperatively, hypoxemia was markedly improved, and was associated with a reduction of the level of pulmonary hypertension, contributing to marked symptomatic improvement.

Tetralogy of Fallot S,D,I: Successful repair without a conduit

Tetralogy of Fallot [S,D,I] is a recently discovered form of tetralogy with solitus atria (S) and D-loop ventricles (D), but with inverted normally related great arteries (I). Because of infundibuloarterial inversion, the right coronary artery must traverse the stenotic pulmonary outflow tract, increasing the risk of surgical trauma to the right coronary artery. Here we report a case of successful surgical repair of tetralogy of Fallot [S,D,I] without the use of an extracardiac conduit.

胸骨縦切開による開心術後乳びろうの１手術治験例

胸骨縦切開による開心術後乳びろうの１手術治験例

A child case of successful surgical repair for tracheal injury secondary to blunt chest trauma

A child case of successful surgical repair for tracheal injury secondary to blunt chest trauma

Successful surgical management of left ventricular free wall rupture in the course of myocardial infarction

The case of a 49-year-old patient is described who presented with cardiogenic shock and electrocardiographic signs of an inferolateral Q-wave infarction, and who received systemic lysis with anisoylated plasminogen streptokinase activator complex (Eminase). After coronary angiography had revealed only peripheral occlusion of a posterolateral branch of the left circumflex coronary artery, a pericardial effusion surrounding both right and left ventricular cavity was identified by echocardiography and was successfully drained via an inferior pericardiotomy with an immediate rise of blood pressure. Upon thoracotomy myocardial rupture was detected in the infarct area and was closed with mattress sutures. A total of 39 cases of successful surgical repair of myocardial free wall rupture reported in the literature is discussed. The mean age of patients was 59.6 +/- 1.3 years. Posterior and anterolateral infarctions were the preferred locations of myocardial rupture. Rupture occurred with a mean delay of 5.0 +/- 1.0 days after the onset of clinical infarct signs. Among patients saved by surgical means were 33 males and 6 females.

Combined septal perforation and cardiac rupture after myocardial infarction: Clinical features and surgical considerations of a correctable condition

The combination of septal perforation and cardiac rupture after myocardial infarction has rarely been reported. This article describes two cases of successful surgical repair of this condition and reviews another three cases previously reported in the literature. The important clinical features were as follows: (1) acute occlusion of a single dominant coronary artery; (2) renewed chest pain before perforation or rupture; (3) electromechanical dissociation at the time of frank rupture; (4) rupture through the left ventricular free wall; (5) in three patients both complications occurred within 3 days of the infarction, and in the other two patients they developed within three weeks. The main surgical considerations are as follows: (1) femoral cannulation to prevent exsanguination before instituting cardiopulmonary bypass; (2) electrically induced fibrillation to avoid systemic embolism; (3) a left ventricular approach to repair the septal perforation followed by infarctectomy; (4) concomitant revascularization was unnecessary. In conclusion, combined septal perforation and cardiac rupture after myocardial infarction is a potentially correctable condition that requires early diagnosis and prompt surgical intervention.

91 Aorto-esophageal fistulaの一手術治験例

91 Aorto-esophageal fistulaの一手術治験例