Coincidence of Total Anomalous Pulmonary Venous Drainage to the Superior Vena Cava,Common Atrium,and Single Ventricle: A Very Rare Condition

Single ventricle defects, including inflow emptying into 1 ventricle, occur at an incidence of 0.05–0.1 per 10,000 live births. The association of total anomalous pulmonary venous drainage (TAPVD) with single ventricle defects is a risk factor for poor outcome. Conversely, common atrium, a rare congenital anomaly, is an endocardial cushion defect. We present a rare case of an unoperated patient with coincidence of a single ventricle defect, TAPVD with direct drainage from the pulmonary veins into the superior vena cava, and common atrium. To the best of our knowledge, such a case has not been previously reported in the literature.     

Added value of three‐dimensional transthoracic echocardiography in assessment of an adult patient with atrioventricular septal defect

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three‐dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en‐face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three‐dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography.     

Left ventricular outflow tract obstruction in atrioventricular septal defects: a pathologic and morphometric evaluation.

Subaortic stenosis has been described with increasing frequency as an ominous feature of atrioventricular septal defect (AVSD), especially following surgical correction of the anomaly in non-Down's syndrome patients. In order to study the surgical anatomy of the left ventricular outflow tract in this malformation, 48 hearts featuring AVSD were examined. Obstructive lesions were classified into unequivocal forms (class A, 13.5%) and potential ones (class B, 10.8%). In the remaining hearts (class C, 75.7%) no obstruction was noted. In class A, subaortic stenosis was due to exaggeration of the anticipated anomalous arrangement of atrioventricular valve tensor apparatus, to the persistence of a subaortic muscular infundibulum, and to a discrete fibrous diaphragm. A potential for subaortic stenosis is provided by the unwedged position of the aortic valve. The left ventricular outflow tract is transformed into a long, forward-displaced fibromuscular channel. Morphometric analysis showed in AVSD (with both common annulus and separate orifices) a significantly (p less than 0.01) lower inflow/outflow tract ratio, and a significantly (p less than 0.01) lower right ventricular/left ventricular outflow length ratio than normal hearts. These results suggest that AVSD is characterized not only, as commonly stated, by inflow tract shortening, but by outflow tract lengthening as well. On these anatomical grounds, nearly all cases of AVSD could harbor the potential for subaortic stenosis; however, this becomes a real hazard (class B) only when associated with forward displacement of the left anterior papillary muscle, or direct insertion on the ventricular septum of the anterior bridging leaflet, and it may be converted to an actual obstruction by the effects of surgery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Gargantuan Left Atrium: A Sequela of Mitral Regurgitation and Mitral Stenosis

Cardiac magnetic resonance imaging and echocardiography revealed a gargantuan left atrium measuring 18.9 cm × 15.7 cm × 11.3 cm in a 56‐year‐old patient diagnosed with severe rheumatic mitral stenosis, severe pulmonary hypertension, and permanent atrial fibrillation. A chest x‐ray also revealed a cardiothoracic ratio approaching 1.0 and a transthoracic echocardiogram measured diameters as large as 19.2 cm. The patient then underwent mitral valve replacement and left atrial reduction surgery and has had no further admissions or complications.     

Rupture of a Noncoronary Sinus of Valsalva Aneurysm into the Left Atrium: A Rare Cause of Acute Pulmonary Edema

A sinus of Valsalva aneurysm is a dilatation of the aortic wall caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve. It has an incidence of <0.1%. The most common cause of a sinus of Valsalva aneurysms is congenital, although they may also be acquired. The most common complication is rupture into the right atrium or ventricle, with rupture into the left chambers occurring very rarely. We present a 40‐year‐old man admitted to the hospital with an acute onset of respiratory distress and pleuritic chest pain. Transthoracic echocardiography followed by transesophageal echocardiography showed rupture of a noncoronary aneurysm of Valsalva sinus into the left atrium. The jet from the fistula caused retrograde flow into the pulmonary veins.     

Ebstein's Anomaly with Subpulmonary Obstruction– A Rare Association

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3‐year‐old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.     

Giant right atrium and subvalvular pulmonary stenosis: A case report of an interesting combination

A 20‐year‐old Congolese woman presented with presyncope, dyspnea, and anasarca. Past medical history was unremarkable. Echocardiography revealed a rare combination of giant right atrium (RA), a dilated and hypertrophied right ventricle, subvalvular pulmonary stenosis (subPS), severe tricuspid regurgitation (TR), pericardial effusion and what appeared to be a spontaneously closed ventricular septal defect (VSD). Cardiac Magnetic Resonance and Cardiac Computed Tomography confirmed the findings excluding the presence of intra‐cardiac and extra‐cardiac shunt and other associated congenital anomalies. The patient underwent subPS resection, right atrioplasty, and tricuspid annuloplasty. Multimodality approach facilitated the detection of the abnormalities and provided clarity when determining the optimal surgical strategy.     

Congenital Pulmonary Venous Stenosis Combined with Valvular Aortic Stenosis: A Very Rare Case

Congenital pulmonary venous stenosis is a rare condition leads to progressive pulmonary hypertension and heart failure. It may be isolated or associated with other cardiac malformations. The condition ranges from hypoplasia of individual pulmonary veins to constriction of the intima of the venoatrial junction. We present a case of congenital PVS involving 4 pulmonary veins combined with severe valvular and subvalvular aortic stenoses.     

Multiplane Transesophageal Echocardiographic Detection and Differential Diagnosis of Isolated Right Pulmonary Artery Agenesis

We present a case report of isolated right pulmonary artery agenesis detected by multiplane trans-esophageal echocardiography (TEE). A special emphasis is placed on its important role in the differential diagnosis and hemodynamic and functional evaluation of patients with unilateral pulmonary oligemia on chest radiograph. We also present a case report of pulmonary embolism with complete obstruction of the right pulmonary artery for better understanding and for comparison of TEE images in patients with pulmonary artery agenesis.     

Transcatheter Closure of a Right Pulmonary Artery to Left Atrium Fistula Using a Ventricular Septal Defect Occluder

Background: Communication between the right pulmonary artery (RPA) and left atrium (LA) is a rare cause of central cyanosis in pediatric patients. Case presentation: We describe a 3-year-old female patient with an oxygen saturation of 70% at admission. The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization. The angiography of pulmonary artery revealed a 7.4 mm × 7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion. Conclusion: The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect. It should be considered in differential diagnosis of cyanosis in children.     

原发性高血压左房功能评价及其临床意义

正常的左房功能对维持高血压病患者的左室功能十分重要。左房功能主要包括蓄储功能、管道功能和辅泵功能。研究表明,高血压病患者左心房功能改变表现为左心房容量增加、收缩期泵血功能增加及左房被动排空功能降低。年龄、左心室重量指数、左心室构型、左房收缩前容积指数为影响左心房射血力的重要因素。因此,评价左房功能有助于临床早期诊断左室功能不全。     

A Rare Case of Permanent Junctional Reciprocating Tachycardia Ablated on the Roof of the Left Atrium

A Rare Case of Permanent Junctional Reciprocating Tachycardia. Left‐sided anteroseptal accessory pathways that course through the aortomitral fibrous continuity are some of the rarest types of accessory pathways. At this region the atrium and ventricle are separated by their greatest distance because of the intervening aortic valve. These pathways often have a long circuitous course that may involve the root and cusps of the aortic valve. Prior reports have demonstrated the feasibility of ablating these pathways along the anteroseptal mitral annulus, the left ventricular outflow tract, or in the left or noncoronary cusps of the aortic valve. We describe a case of a concealed decremental anteroseptal accessory pathway that was ablated on the roof of the left atrium remote from the mitral or aortic valve annuli. This report indicates that when an appropriate site for ablation of a left‐sided anteroseptal accessory pathway is not found close to a valve annulus, these pathways may be amenable to ablation by targeting their atrial insertion site. (J Cardiovasc Electrophysiol, Vol. 24, pp. 464‐467, April 2013)     

A Unique Case of Co-Occurrence of Bilateral Pulmonary Artery Embolism and Right Atrium Thrombus

Pulmonary embolism (PE) is considered a major cause of morbidity and mortality in both inpatient and outpatient settings. PE can be presented in different clinical pictures. However, the association between PE and right atrial thrombus is not frequently reported. PE is categorized based on size, location and clinical presentation. Although management changes between different PE sub-types, optimal medical therapy is not well established for coexisting PE with right atrial thrombus. Herein, we present a unique case of a 58-year-old male with bilateral PE and atrial thrombus, with no known underlying risk factors.     

Percutaneous edge‐to‐edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome,single ventricle physiology,and unbalanced atrioventricular septal defect

Congenital heart disease patients, specifically with unbalanced atrioventricular septal defects and common atrioventricular valves requiring single ventricle palliation, have substantial morbidity and mortality. Atrioventricular valve regurgitation (AVVR) is associated with poor outcomes in single ventricle patients, and many of them require surgical treatment of AVVR in their lifetimes. We describe a unique case of transcatheter edge‐to‐edge valve repair using the MitraClip system (Abbott, Chicago, IL) in a single ventricle patient with severe common AVVR.     

Atrioventricular block: a serious complication in and after transcatheter closure of perimembranous ventricular septal defects

BACKGROUND: Transcatheter closure is an effective approach for perimembranous ventricular septal defects (PMVSD). However, atrioventricular blocks (AVB) emerged possibly due to the close proximity of the PMVSD to the conduction system, but concern for the complication was not adequately emphasized. In this study, we report the incidence of AVBs, in and after transcatheter closure of a PMVSD, and the outcome of the complication in our center. METHODS: One hundred and sixty-eight PMVSD patients were accepted for transcatheter closure with Amplazter PMVSD occluder (AGA Medical, Plymouth, Minn., USA). The procedure was discontinued when a second- or third-degree AVB occurred. A steroid was administered to all patients who developed AVBs. Temporary pacemakers were inserted in patients who developed a complete AVB or Mobitz type II AVB during or after the procedure. RESULTS: During the follow-up period of 6-24 mo (mean 10.6 +/- 3.9), the incidence of AVBs occurring during or after transcatheter closure of PMVSD was 3.5%. The AVB disappeared quickly after discontinuing the procedure in patients who developed AVBs during the procedure, whereas the AVBs disappeared between 2 and 21 d (mean 8.0 +/- 8.8) in the patients who developed AVBs after the procedure. However, complete right bundle branch block (CRBBB) was observed, and a transient complete AVB emerged after 8 mo in 1 case, incomplete right bundle branch block (IRBBB) in 1 case, and CRBBB and left anterior hemiblock (LAH) in 1 case. CONCLUSIONS: The AVB is a serious complication during and after transcathter closure of PMVSD. More attention should be paid to the complication, and multicentres are required to monitor the complication.