Diagnosis and Surgical Treatment of Congenital Cor Triatrium

Objectives To evalu-ate the outcome of diagnosis and surgical treatment for cor triatrium (CTA) in 6 patients seen between 1994 and 2002. Methods 6 patients ranging in age from 5 months to 25 years were observed. All of them had other cardiovascular defects, and presented with dyspnea, palpitations (and low weight growing only in the infants) . Preopreative two dimensional echocardiogra-phy had demonstrated an abnormal septum in the left atrium and other coexistent anomalies. In 3 of them the membrane was obstructed between the left atrial accessory chamber and the left atrium. The communication were atrial septal defect (ASD) indirectly; The clinical findings were due to the pulmonary hypervas-cularity. Only one case had a fenestration in the septum with a small patent foramen ovale (FO) directly , and the clinical findings were due to the obstruction to flow through the membrane in the left atrium, producing venocapilar pulmonary hypertension. Two of them had ASD and fenestration on the septum. The ot     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Cor Triatriatum

Cor triatriatum is defined as a membrane within the left atrium, which might lead to restricted pulmonary venous return. Diagnosis is usually achieved by echocardiography, therapy of choice is excision of the membrane. Upon ten new cases, the association with other congenital heart diseases (CHDs), clinical symptoms and the surgical approach are discussed. Eight of ten patients were children, six of them aged<1 year. Additional CHDs included atrial and ventricular septal defects, partial anomalous pulmonary venous return and complex CHD. Surgery was performed in all cases. Prognosis is related to associated CHD.     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage,TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例,心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口,9例采用心上法,后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁,自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例,其他病人痊愈出院,术后随访3个月-4年,恢复良好,无吻合口狭窄发生,出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要,手术治疗的关键在于左心房与肺总静脉吻合口应足够大,避免狭窄的发生。     

A case of abnormal venous drainage of the whole left lung into the left innominate venous trunk

In this case, the drainage of all the left lung veins of gathered in a common trunk into the left innominate venous trunk was combined with an atrial septal defect and partition of the left atrium. The rarity of such cases in the literature is due to the fact that it is an uncommon anatomical anomaly and that very often it is symptomless, and therefore does not induce the subjects with this anomaly to seek medical advice. In the case reported, surgical treatment consisted in closing the atrial septal defect, resecting the abnormal left atrial partition and in re-establishing the drainage of the left lung veins into the left atrium through a wide latero-lateral anastomosis with ligature of the abnormal left pulmonary venous trunk as its junction with the left innominate venous trunk.     

Cor triatriatum: study of 20 cases.

Twenty cases of cor triatriatum are reported. The diagnosis was confirmed by necropsy in 16 cases and at the time of operation in 4. The lesion occurred as an isolated anomaly in 7 cases; in 13, other associated cardiac anomalies were present. Three anatomic types of cor triatriatum were identified in the cases studied at necropsy: diaphragmatic (10 cases), hourglass (3) and tubular (3). The diaphragmatic type was also present in all four cases in which the diagnosis was confirmed at operation. Associated anomalies were found in five cases of the diaphragmatic type and in each case of the hourglass of tubular types. In isolated cor triatriatum the clinical findings were characteristic of pulmonary venous and arterial hypertension. In two cases, one with a communication between the right atrium and the accessory left atrial chamber and one with partial anomalous pulmonary venous connection associated with cor triatriatum, the clinical findings suggested a large left to right shunt with pulmonary arterial hypertension. The clinical findings varied in the cases with associated anomalies, and it was difficult to determine the cause of disturbance of the circulation.     

Bronchogenic cyst causing atrial fibrillation by impinging the right inferior pulmonary vein

Atrial fibrillation is the most common sustained cardiac arrhythmia and is usually associated with underlying structural heart disease, but may also occur in isolation--the entity of "lone" atrial fibrillation. Recently, attention has been directed to the pulmonary veins as a source of the arrhythmia through identification of rapidly firing ectopic foci within the covering myocardial sleeves. We describe a 38-year-old man who presented with treatment-resistant atrial fibrillation and a posterior mediastinal mass. Electrophysiological studies demonstrated abnormal foci of electrical activity at the entrance of the right inferior pulmonary vein into the left atrium. Surgical exploration revealed a bronchogenic cyst that distorted and stretched the right inferior pulmonary vein as it traversed the posterior mediastinum towards the left atrium. Restoration of sinus rhythm without recurrence of atrial fibrillation characterized the clinical course after surgical resection of the mass. This case demonstrates that a retro-cardiac bronchogenic cyst can cause atrial fibrillation by impinging on a pulmonary vein.     

Mitral atresia with normal aortic valve: a study of eighteen cases and a review of the literature.

Eighteen cases of mitral atresia with normal aortic valve plus 68 cases from the literature are analyzed. A new classification based on anatomical findings is proposed. Pulmonary stenosis or atresia is frequent in the type with transposition of the great arteries. The rare instances of normal or large left ventricle are due to a large ventricular septal defect, or to straddling or displaced tricuspid valve. The clinical, radiologic and electrocardiographic findings have been summarized and correlated with the different physiopathological situations. In our catheterized cases an oxygen saturation step-up was found in the right atrium together with left atrial hypertension. In seven cases mitral atresia was demonstrated by selective left atrial contrast injection. The average age at death was six months. Among the cases surviving one year or more, the association of atrial septal defects or pulmonary stenosis was frequent. Enlargement of the interatrial communication, accompanied by other palliative measures, is the only available surgical procedure.     

Atresia of the left atrioventricular valve with patency of the aorta: anatomico-functional analysis of 23 patients.

We analyzed the findings in 23 patients with atresia of the left atrioventricular valve and a patent aorta seen in the period from January 1980 to July 1989. Having divided the cases according to the anatomical findings, we made a subsequent analysis of the clinical and surgical results with the aim of establishing the management most likely to diminish risks, still high, in treatment of this complex anomaly. From the anatomical viewpoint, three variants were observed. In the first, made up of 15 cases, there was absence of the left atrioventricular connexion. The characteristic finding in the second group, with five cases, was an imperforate left atrioventricular valve in the setting of concordant atrioventricular connexions. The third group, of these cases, was dominated by the presence of isomerism of the atrial appendages, both appendages being of left morphology in one case, and of right morphology in the other two. Further anatomical variation was then found in each group. Nine of the 15 with absence of the left atrioventricular connexion had the right atrium connected to a dominant left ventricle in presence of a rudimentary and incomplete right ventricle associated with discordant ventriculo-arterial connexions, all of them being in usual atrial arrangement and three with pulmonary stenosis. The remaining six in this first variant had the right atrium connected to a dominant right ventricle. In the five patients with imperforate left atrioventricular valves, two had discordant and three had concordant ventriculo-arterial connexions. In the three cases with isomerism, two had absence of the left atrioventricular connexion, with a dominant right ventricle. The last patient had an imperforate left atrioventricular valve and a discordant ventriculo-arterial connexion. From the functional viewpoint, there were 14 patients (10 with absence of an atrioventricular connexion, four with imperforate atrioventricular valve) with congestive heart failure and nine patients (five from the first, one from second, and three from the third variant) with hypoxia. Long-term follow-up (median 16.4 months--varying from 1 to 41 months--in the group with congestion and 27.7 months--varying from 12 to 57 months--in those with hypoxia) showed favorable clinical evolution in 11 (91%). We conclude that an anatomico-functional division can point towards the most appropriate management in this complex anomaly.     

Catheter directed embolectomy of right atrial clot in transit-A case series

In the previous literature, the prevalence of right atrial (RA) clot-in-transit associated with pulmonary embolism is around 4–18% with an associated mortality of 80–100% in untreated cases. Surgical thrombo-embolectomy has been the mainstay of treatment for stable patients but the data for percutaneous thrombo-embolectomy is lacking in the literature. We present a series of three cases of right atrium clot-in-transit treated with catheter-based therapies with Inari FlowTriever® (Inari Medical, Irvine, CA). Our three patients had different clinical profiles and presentations of right atrial clot-in-transit. All of the subjects had contraindications to surgical thrombo-embolectomy and thrombolytic therapy. Catheter based embolectomy using Inari FlowTriever® was successfully performed in all the patients. As the data on this intervention is sparse, our case series highlights successful catheter based thrombo-embolectomies in high-risk individuals with right atrial clot in-transit with or without pulmonary embolism.     

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment

Background and purposeSinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium.Summary of caseWe report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect.ConclusionsInterventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.     

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.     

Left atrial thrombus causing pulmonary embolism by passing through an atrial septal defect.

A 66-year-old woman admitted with dyspnea on exertion had atrial fibrillation and left ventricular dysfunction. Echocardiography revealed an atrial septal defect (ASD) and a soft, easily deformable thrombus in the dilated left atrium. The atrial mass suddenly disappeared on the 10th day after admission, and contrast-enhanced chest computed tomography and pulmonary blood flow scintigraphy showed that the thrombus had detached from the left atrium, floated into the right atrium through the ASD and caused pulmonary embolism. This is the first documented case of a left atrial thrombus causing pulmonary embolism by passing through an ASD. When an ASD is present, it is important to consider not only paradoxical thromboembolism (from the right to the left atrium), but also pulmonary embolism caused by thromboembolism from the left to the right atrium.     

Identification and ablation of atypical atrial flutter. Entrainment pacing combined with electroanatomic mapping

Differentiation between typical and atypical atrial flutter solely based upon surface ECG pattern may be limited. However, successful ablation of atrial flutter depends on the exact identification of the responsible re-entrant circuit and its critical isthmus. Between August 2001 and June 2003, we performed conventional entrainment pacing within the cavotricuspid isthmus in 71 patients with sustained atrial flutter. In patients with positive entrainment we considered the arrhythmia as typical flutter and treated them with conventional ablation of the cavotricuspid isthmus. As a consequence of negative entrainment we performed 3D-electroanatomic activation mapping (CARTO trade mark ). Conventional ablation of the right atrial isthmus was successful in all patients (n = 54) with positive entrainment. We performed electroanatomic mapping in the remaining 17 patients (14 male; age 60.9 +/- 16 years) resulting in the identification of 6 cases with typical and 11 cases with atypical flutter. Therefore, entrainment pacing was able to predict the true presence of typical atrial flutter in 91.5%. Atypical flutter was right sided in 4 patients and left sided in 7 cases. Electrically silent ("low voltage") areas probably demonstrating atrial myopathy were identified in all cases with left sided and in 2 patients with right sided flutter. In these patients targets for ablation lines were located between silent areas and anatomic barriers (inferior pulmonary veins, mitral respectively tricuspid annulus, or vena cava inferior). In 1 patient, the investigation was stopped due to variable ECG pattern and atrial cycle lengths. In the remaining cases, ablation was acutely successful. One patient, after surgical closure of a ventricular septal defect, demonstrated a dual-loop intra-atrial reentry tachycardia dependent on two different isthmuses. This arrhythmia required ablation of those distinct isthmuses to be interrupted. After a mean follow-up of 8.8 +/- 3.4 months, there was one patient with a recurrence of left-sided atrial flutter. Another patient developed permanent atrial fibrillation shortly after the procedure. Mean duration time of the procedure was 235.6 +/- 56.4 min (right atrium: 196 +/- 17.3 min; left atrium: 267.2 +/- 59.5 min), and average fluoroscopy time was 21.8 +/- 11.7 min (right atrium: 9.5 +/- 6 min; left atrium: 28.9 +/- 7 min). There was no incidence of serious complications associated with these procedures. In conclusion, conventional pacing in the cavotricuspid isthmus combined with electroanatomic mapping was an effective method to differentiate between typical and atypical atrial flutter. Electroanatomic mapping was a powerful tool both for identification of different atrial re-entrant circuits including their critical isthmuses as well as for effective application of individual ablation line strategies.     

非接触心内膜激动标测系统引导下的左房消融治疗阵发性心房颤动的初步研究

为探讨非肺静脉起源的阵发性心房颤动 (简称房颤 )用非接触心内膜激动标测系统 (EnSite30 0 0 )标测房颤的起始激动部位、折返途径与传导以及射频消融术治疗的方法、效果及其安全性。对 6例左房起源的房颤 ,用改良Ross法穿刺房间隔后置入EnSite30 0 0标测球囊导管于左房 ,将大头导管分别送至左上、下肺静脉 ,右上、下肺静脉 ,二尖瓣口及在左房前后、左右各壁移动 ,描记左房三维几何图形。记录诱发房颤的房性早搏 (简称房早 )起源点、房颤起始的传导方向、折返部位。设计消融点与消融线 ,用 5 0～ 5 5℃温控消融导管放电 ,每点 6 0s。参考消融终点 :①房早等房性心律失常消失 ,必要时静脉滴注异丙肾上腺素重复检查 ;②设计的线状消融部位传导中断 ;③先前的电生理方法不能诱发房颤和其他房性心律失常。结果 :经EnSite30 0 0标测 ,6例房早均起源于左房后壁 ,其中单点起源 2例、多点起源 4例 ;折返限于左房壁 2例、与左上肺静脉有关 3例、与右上肺静脉有关 2例、合并典型心房扑动 1例。EnSite30 0 0引导下的左房消融术 3例成功、3例有效 ,无并发症发生。随访 1个月 ,2 4h动态心电图检查术后房早明显减少 (5 6 .8±7.5 8个vs 15 2 6 2 .4± 8914 .5个 ,P <0 .0 0 1)。结论 :用EnSite30 0 0能准确标测左房非?     

改良左心房上吻合法矫治心上型完全性肺静脉异常回流

目的总结从左心房顶部行改良左心房-共同静脉吻合(左心房上吻合法)治疗心上型完全性肺静脉异常回流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2005年1月至2011年8月高州市人民医院收治心上型TAPVC 17例,17例肺静脉均是经垂直静脉、无名静脉、右上腔静脉后回流至右心房,其中男7例,女10例,年龄中位数为1岁(40 d～24岁);体质量中位数为12.5 kg(3.1～31.0 kg),全组12例合并房间隔缺损,3例合并卵圆孔未闭,2例合并房间隔缺损及动脉导管未闭,均采用左心房上吻合法。结果手术死亡2例,1例死于低心排血量综合征;1例术后反复肺感染,死于呼吸衰竭。1例因术后急性肺水肿,二次手术重新开放垂直静脉延迟闭合治愈;2例心律失常(频发房性期前收缩),治疗一周恢复正常。无肺动脉高压危象。术后随访3个月至6年,平均3.5年,无吻合口狭窄和心律失常,发绀消失,活动耐力显著增加,生长发育良好。结论改良左心房上吻合法治疗心上型TAPVC,手术暴露好、操作简单、心律失常发生率低,术后中期随访结果良好。     

Secretion of atrial natriuretic peptide from the heart in man

Plasma concentrations of atrial natriuretic peptide were measured in eight patients undergoing elective cardiac catheterisation and angiography. All patients had normal resting pressures in the cardiac chambers and no clinical evidence of heart failure. Plasma atrial natriuretic peptide rose significantly from the superior vena cava into the right atrium and right ventricle. The increase into the right atrium was variable, with no increase in three subjects, but there was a consistent increase in all subjects from the superior vena cava to to the right ventricle. These findings in the right atrium are probably caused by inadequate mixing and streaming of blood from the coronary sinus containing high concentrations of atrial natriuretic peptide. There was no increase in the concentration of natriuretic peptide from the pulmonary artery to the left ventricle, but the concentrations in the left ventricle were significantly higher than in the superior vena cava. These findings demonstrate that the heart secretes atrial natriuretic peptides in the absence of cardiac failure. Studies based on sampling of the right atrium will not accurately measure cardiac secretion of atrial natriuretic peptide and will therefore be likely to obscure the mechanisms responsible for regulating its secretion. The right ventricle and pulmonary artery are the best sampling sites to measure atrial natriuretic peptide release from the right atrium.     

Unusual drainage of the inferior caval vein in left atrial isomerism

Two of the 26 cases of left atrial isomerism in our clinical material had an unusual drainage of the inferior caval vein. In one, the inferior caval vein crossed from left to right infradiophragmatically and then drained into the right-sided morphologically left atrium. In the second case, there was azygos continuation to both right and left superior caval veins. These variations have major surgical significance.     

Secretion of atrial natriuretic peptide from the heart in man.

Plasma concentrations of atrial natriuretic peptide were measured in eight patients undergoing elective cardiac catheterisation and angiography. All patients had normal resting pressures in the cardiac chambers and no clinical evidence of heart failure. Plasma atrial natriuretic peptide rose significantly from the superior vena cava into the right atrium and right ventricle. The increase into the right atrium was variable, with no increase in three subjects, but there was a consistent increase in all subjects from the superior vena cava to to the right ventricle. These findings in the right atrium are probably caused by inadequate mixing and streaming of blood from the coronary sinus containing high concentrations of atrial natriuretic peptide. There was no increase in the concentration of natriuretic peptide from the pulmonary artery to the left ventricle, but the concentrations in the left ventricle were significantly higher than in the superior vena cava. These findings demonstrate that the heart secretes atrial natriuretic peptides in the absence of cardiac failure. Studies based on sampling of the right atrium will not accurately measure cardiac secretion of atrial natriuretic peptide and will therefore be likely to obscure the mechanisms responsible for regulating its secretion. The right ventricle and pulmonary artery are the best sampling sites to measure atrial natriuretic peptide release from the right atrium.     

Unusual growth of a left atrial myxoma complicated by a secundum atrial septal defect.

A 55 year-old man had a myxoma that originated in the left atrium and grew through a secundum atrial septal defect into the right atrium. The tumor, which was attached by a pedicle to the lateral wall of the left atrium near the right pulmonary vein, was resected under cardiopulmonary bypass. Transesophageal echocardiography was important in the successful outcome of surgical treatment.