Pseudoaneurysm following modified Blalock-Taussig shunt mimicking suprasternal abscess: A rare presentation

The modified Blalock-Taussig shunt is a well-recognized palliative procedure for cyanotic congenital heart disease. Post procedure pseudoaneurysm mimicking a suprasternal abscess is of very rare occurrence. We present a child of L-Transposition of great arteries (L-TGA), pulmonary atresia and ventricular septal defect (VSD) with small pulmonary artery size that underwent modified Blalock Taussig shunt for severe cyanosis at 1 1/2 years. Six months after the procedure the child presented with a suprasternal swelling and fever. Angiography showed pseudoaneurysm from the proximal anastomotic site. The pseudoaneurysm was suitably tackled by interventional techniques and surgery for the underlying cardiac condition is being planned.     

Cardiac Valve Papillary Fibro-Elastoma: a Report on Three Different Clinical Cases

Cardiac papillary fibro-elastoma is a rare benign endocardial tumour predominantly affecting the aortic and mitral valves. We report three cases with different clinical presentations, and discuss the diagnostic and therapeutic strategies.     

继发于先天性心脏病术后的主动脉瓣下狭窄手术矫治分析

目的 总结少见病例——继发于先天性心脏病（congenital heart disease,CHD）手术后主动脉瓣下狭窄（secondary subaortic stenosis,SSS）的手术疗效,并分析导致SSS发生的原因。 方法 回顾性分析2008年6月1日至2012年12月31日期间广东省人民医院对SSS进行手术矫治19例患者的临床资料,其中男10例,女9例。CHD类型主要包括右心室双出口、室间隔缺损、部分性房室间隔缺损等。患者矫治CHD时的年龄：4个月（15 d～5岁11个月）;行主动脉瓣下狭窄矫治时的年龄：5岁3个月（1岁～15岁3个月）。患者行SSS矫治与CHD矫治的相隔时间：4年10个月（8个月～13年11个月）。 结果 所有患者均行主动脉瓣下狭窄矫治术,无术中死亡;体外循环时间79 （39～172） min,主动脉阻断时间42 （22～124） min。术后住院时间7 （5～9） d。1例术后需安装永久性心脏起搏器。所有患者均随访。随访时间1年10个月（5个月～4年4个月）。所有SSS患者术后无临床症状,心功能正常,随访过程中无死亡。1例患者随访过程中左心室流出道狭窄逐渐加重,再次行主动脉瓣下狭窄矫治术。 结论继发先天性心脏病术后出现主动脉瓣下狭窄极少见,其发生原因考虑CHD矫治后左心室流出道血流动力学紊乱造成,其发生时间与继发先心病手术类型无法预测,主动脉瓣下狭窄矫治术对SSS矫治简单、有效,但术后左心室流出道仍有可能再次狭窄并加重。     

多心腔多发粘液瘤的临床特征与手术方法

目的　探讨多心腔多发粘液瘤的临床特征,改进其手术方法,避免术中瘤体遗漏,心肌损伤,提高手术生存率。　方法　收治多心腔多发粘液瘤１２ 例。在体外循环下切除瘤体３３ 个。术后长期观察随访。　结果　术中发现超声诊断对该症往往漏诊,原手术方法对心肌损伤大,易出现并发症。术后５ 例复发,其中３ 例因拒绝和等待手术者死于脑栓塞;另２ 例再手术者死亡１ 例,８ 例健在。　结论　本病手术难度大,术中要仔细探查,避免遗漏。术后复发率高,要长期随访。该症瘤体更易脱落,一旦确诊应及早手术。     

Right atrial paraganglioma: an unusual primary cardiac tumour

We present a case of an unusual presentation of a rare primary cardiac tumour. There are no more than 50 previously reported cases of primary cardiac paraganglioma in the world literature and only a small proportion of these involve the right side of the heart. Diagnosis is difficult and surgical management is varied and complex.     

Cardioprotection by breathing hyperoxic gas—relation to oxygen concentration and exposure time in rats and mice

Objectives: Breathing a hyperoxic gas (≥95% O₂) protects against ischaemia-reperfusion injury in rat and mouse hearts. The present study investigated how oxygen concentration and duration of hyperoxic exposure influenced cardioprotection, and whether hyperoxia might induce delayed cardioprotection (after 24 h). Methods: Animals were kept in normal air or in a hyperoxic environment, and their hearts were isolated and Langendorff-perfused immediately or 24 h thereafter. Global ischaemia was induced for 25 min in rats and 40 min in mice, followed by 60 min of reperfusion. Infarct size was determined by triphenyl tetrazolium chloride staining. Results: In rats exposure to ≥95, 80, and 60%, but not to 40% of oxygen immediately before heart isolation and perfusion improved postischaemic functional recovery. Eighty or more percent of oxygen also reduced infarct size. A preconditioning-like effect could be evoked by 60 or 180 min of hyperoxia, giving both immediate and delayed protection. In the mouse heart protection could be induced by pretreatment for 15 or 30, but not by 60 min with ≥95% oxygen. The protective effect of hyperoxia in mice could be evoked in the immediate model only. Conclusions: Hyperoxia protects the isolated rat and mouse heart against ischaemia-reperfusion injury, but some species-different responses exist. The protection depends on both oxygen concentration in inspired air, and duration of hyperoxic exposure.     

Heart valve surgery

Heart valve surgery has made significant advances over the last six decades. The development of the heart lung machine in 1953 allowed surgeons to operate on the heart by separating it from the rest of circulation. Treatment of valve disease ranges from transcatheter implantation (TAVI) to full standard operative replacement. In the last decade, there has been a shift towards heart team decision making regarding valve intervention. This enables high-risk patients to be offered life-changing treatment. The heart team should include cardiac surgeons, interventional cardiologists and imaging cardiology specialists. There have been further advancements in minimally invasive repairs and replacements (including percutaneous approaches to valve therapies). Anatomical and physiological knowledge is paramount to the decision making process regarding indications, reparability and outcomes following surgery. This article discusses the anatomy of each valve, the aetiology of the pathological processes and the surgical methods for addressing these disease processes.     

Primary cardiac sarcoma presenting as shock

Primary cardiac sarcomas are rare tumors with an unfavourable prognosis. Complete surgical resection is currently the only mode of therapy proven to show any benefit. We report an adult male who presented to emergency department with shock. Echocardiography revealed a huge right ventricular mass obstructing the right ventricular outflow tract and he underwent emergency resection of the mass. Subsequent histopathology confirmed the diagnosis of fibromyxosarcoma. Primary cardiac sarcoma presenting as circulatory shock is a rare phenomenon.     

Left pulmonary agenesis with single atrium simulating cardiac type of total anomalous pulmonary venous connection

Pulmonary agenesis is rare though not unknown. Factors such as atypical presentation, unknown etiology and frequent misdiagnosis make it a challenging malformation. Familial agenesis has been documented. Left pulmonary agenesis is normal in the snakes. When it occurs in humans, failure of up gradation of genes to the human level is a possibility. An instance of left pulmonary agenesis with an extremely rare association of single atrium, unpublished till date, is reported.     

Left atrial ball thrombus

We report a case of left atrial ball thrombus managed successfully by surgery. This is a rare clinical entity and has been reported quite infrequently in Indian literature despite the high incidence of Rheumatic mitral stenosis. The relevant literature is reviewed.     

Complete avulsion of the papilla of Vater and gastroduodenal artery due to blunt abdominal trauma: Report of a case

A case of traumatic avulsion of the papilla of Vater and gastroduodenal artery successfully treated by pancreaticoduodenectomy is presented herein. The mechanism of this rare injury appeared to be a shearing force applied to the common bile duct and gastroduodenal artery. Thus, when the liver is driven cephalad by compression of the abdomen and by the deceleration force, the common bile duct and gastroduodenal artery are avulsed from the fixed duodenum and pancreas. The mechanism of this rare injury is postulated on the basis of operative and histological findings. Our case is thought to be the first of traumatic avulsion of the papilla of Vater and gastroduodenal artery to be reported in Japan.     

Heart Substitution: Transplantation and Total Artificial Heart. The Texas Heart Institute Experience

Two methods of heart substitution have been used at the Texas Heart Institute: cardiac transplantation and the total artificial heart (TAH). Cardiac transplantation is an effective means of saving patients with endstage heart disease and, since the introduction of the immunosuppressive agent cyclosporine, survival has increased significantly. In a recent series from July 1982 to May 1984, 25 patients underwent cardiac transplantation at the Texas Heart Institute. Nineteen of these patients are alive, in Functional Class I, and leading normal lives. A major disadvantage of cardiac transplantation is that it cannot be used on an emergency basis for postcardiotomy patients. In this situation, TAH has been used twice (1969 and 1981) as the first stage to maintain circulation prior to subsequent cardiac transplantation. In both instances, the TAH functioned well until a satisfactory donor was found. This demonstrates the feasibility of the two-staged concept and encourages further development of cardiac prostheses.     

Structure function interface with sequential shortening of basal and apical components of the myocardial band

Objective: To mechanically test the intact cardiac structure to determine the sequence of contraction within the myocardial mass to try to explain ejection and suction. Methods: In 24 pigs (30–85 kg), segment shortening at the site of sonomicrometer crystals was continuously recorded. The ECG evaluated rhythm, and Millar pressure transducers measured intraventricular pressure and dP/dt. Results: Study of segment shortening defined a sequence of contraction within the myocardial mass, starting at the free wall of the right ventricle and on the endocardial side of the antero-septal wall of the left. Crystal location defined underlying contractile trajectory; transverse in right ventricle followed by basal posterior left ventricle, and from the endocardial anterior wall to the posterior apical segment and finally to the epicardial side of the anterior wall. Mean shortening fraction averaged 18±3%, with endocardial exceeding epicardial shortening by 5±1%. Epicardial segment crystal displacement followed endocardial shortening by 82±23 ms in the anterior wall, and finished 92±33 ms after endocardial shortening stopped, time frame that matches the interval of fast drop of ventricular pressure and the start of suction. Conclusions: Crystal shortening fraction sequence followed the rope-like myocardial band model to contradict traditional thinking, with two starting points of excitation–contraction, the right anterior free wall of the right ventricle, and the endocardial side of the anterior wall. Active suction may be due to active shortening of the epicardial fibers of the anterior wall, because relaxation was not detected when both mitral and aortic valves were closed during the interval previously termed ‘isovolumetric relaxation’.     

Obstructive neonatal atrial myxoma

IntroductionCardiac myxoma is a benign neoplasm representing the most common primary cardiac tumor in adults, however it is unusual in neonates. It is represented by an endocardial mass that occupies the cardiac chamber. Although the majority of myxomas are attached to the fossa ovalis of the interatrial septum, they also attach to the walls of the cardiac chambers and to valve leaflets surfaces. Approximately 75% of myxomas are found in the left atrium, 20% are located in the right atrium, and rarely in the ventricles.Presentation of caseWe describe a rare case of neonatal cardiac myxoma arising from interatrial septum, causing significant mechanical obstruction to blood flow through tricuspid valve, in an otherwise normal newborn. The patient underwent successful excision of the myxoma with an uneventful recovery [1].DiscussionCardiac tumors are rare in children, thus an understanding of the common types of benign and malignant paediatric cardiac tumors and their imaging features, is important because the epidemiology and tumor types differ from those encountered in adults.ConclusionLarge neonatal myxoma is exceptionally rare and even more infrequent is the surgical excision in the first day of life.     

Intussusception secondary to endometriosis of the cecum

INTRODUCTION

Intussusception in adults is a rare cause of bowel obstruction. Endometriosis of the bowel is also a rare entity that can be the cause of bowel obstruction. Here, we report a rare case of intussusception secondary to endometriosis of the cecum.

PRESENTATION OF CASE

A 40-year-old woman presented to the hospital with a one-week history of intermittent epigastric pain. On physical examination, there was a soft, round non-tender palpable mass in the right flank and abdominal computed tomography scan revealed an intussusception. We made the diagnosis of ileo-colic intussusception and performed ileocecal resection. The surgical specimen revealed a round submucosal cystic mass in the cecum and the histology showed endometriosis of the cecum.

DISCUSSION

Intussusception in adults is a rare entity present in just 1% of all patients with bowel obstruction, and 5% of all intussusceptions. In general, intussusception in adults has a pathologic lesion as the lead point and the lesion is a malignancy in 20–50% of the cases. Thus, the treatment of an intussusception in adults should be operative. Endometriosis of the bowel is a rare cause of intussusception. Small endometriosis lesions of the bowel are unlikely to cause symptoms; however, in patients presenting with bowel obstruction, urgent treatment is indicated.

CONCLUSION

Intussusception in an adult is a rare cause of bowel obstruction and intussusception caused by endometriosis is also rare. Although rare, the diagnosis of endometriosis as a cause of intussusception must be considered as part of the differential diagnosis.     

巨大心脏瓣膜置换术的经验

目的 总结65例巨大心脏瓣膜疾病患者的瓣膜置换术的治疗经验。方法 巨大心脏瓣膜疾病患者65例,均在中度低温体外循环下进行手术,采用透析型人工肾超滤技术,心肌保护采用温氧合血停跳液诱导停搏及终末再灌注,4℃冷晶体间断灌注维持停搏技术。单纯二尖瓣置换术45例,均保留瓣下结构(MVR/SVP),主动脉瓣置换术7例,二尖瓣及主动脉瓣联合置换术13例,同时行三尖瓣Devega环缩术20例。结果 全组患者中54例心脏自动复跳,11例电击除颤复跳。3例死亡,2例出现顽固性低心排综合征死亡,1例因凝血功能障碍致创面广泛出血死亡,其余均治愈出院。结论 治疗经验:①充分术前准备;②采用超滤技术;③采用温氧合血停跳液诱导停搏及终末再灌注,4℃冷晶体间断灌注维持停搏心肌保护技术;④采用保留瓣下结构的二尖瓣置换术(MVR/SVP)。     

Bioprosthetic heart valves--replacing order with chaos: electron microscopic study

Rheumatic heart disease is a significant clinical entity in young children, especially in the developing world. One of the major long-term effects of ill managed rheumatic fever is irreversible damage to the cardiac valve leaflets, primarily on the left side. With the limited success of currently available mechanical and bioprosthetic valves, there is an urgent need for new directions in bioprosthetic valves, both in material, including source, degree of fixation, surface, bulk modifications, etc., and design. In the present paper, new proposals in the material selection and fabrication of bioprosthetic valves are proposed based on electron microscopic studies of natural valve leaflets and the pericardial surface. Current approaches for bioprosthetic valve fabrications include the wide use of the pericardium as a leaflet material. The present study indicates a need for nondestructive surface examination of pericardial sheets for the elimination of areas of surface voids resulting from gross fiber disorientation. Also, there seems to be a need for incorporation of an in situ fiber renewal mechanism in bioprosthetic leaflets to emulate the natural valve more closely. Apparently natural leaflets have built-in fiber renewal mechanism(s).     

Heart Retransplantation

Retransplants comprise only a small minority (3-4%) of heart transplants, however outcome following retransplantation is compromised. Risk factors for a poor outcome following retransplantation include retransplantation early (<6 months) after primary transplant, retransplantation for acute rejection or early allograft failure, and retransplantation in an earlier era. The incidence of rejection and infection is similar following primary transplant and retransplantation. The compromised outcomes and risk factors for a poor outcome are similar in adult and pediatric heart retransplantation. However, due to the short half-life of the transplanted heart, it is an expectation that patients transplanted in childhood may require retransplantation. Based on the data available and the opinion of the working group, indications for heart retransplantation are (i) chronic severe cardiac allograft vasculopathy with symptoms of ischemia or heart failure (should be considered) or asymptomatic moderate or severe left ventricular dysfunction (may be considered) or (ii) chronic graft dysfunction with symptoms of progressive heart failure in the absence of active rejection. Patients with graft failure due to acute rejection with hemodynamic compromise, especially <6 months post-transplant, are inappropriate candidates for retransplantation. In addition, guidelines established for primary transplant candidacy should be strictly followed.     

心脏空搏下心内直视手术在先天性心脏病中的应用

目的 探讨浅低温体外循环心脏空搏下心内直视手术在先天性心脏病中应用的可行性。方法 在181例常见的先天性心脏病中,采用这一手术方法,观察该方法对心肌保护的效果,总结气栓防、术野显露及体外循环相关的经验。结果 181例手术顺利,术野显露达到要求,无气栓发生,术后死亡1例。结论 浅低温体外循环心脏空搏下心内直视手术在常见的先心病中具有实用价值,但对体外循环提出了新的要求。