Successful implantation of leadless pacemaker in a patient with giant right atrium and tricuspid valve stenosis

With the steadily increasing amount of leadless pacemaker implantations performed worldwide, it has called attention to the delivery difficulty in patients with severe large right heart. Nevertheless, limited studies have reported leadless pacemaker implantation in patients with tricuspid stenosis. Here, we report the successful implantation of leadless pacemaker in a 60‐year‐old female patient with giant right atrium and tricuspid valve stenosis. It is highlighted that leadless pacemaker should not be discouraged even if there are tricuspid valve stenosis and giant right atrium.     

Surgical treatment of recurrent subvalvular discrete membrane and left ventricular outflow tract stenosis in an adult patient with a history of congenital cardiac surgery

Left ventricular outflow stenosis can develop at the supravalvular, valvular, and subvalvular levels. Resection of strictures at the diffuse subvalvular level is very difficult. In such pathologies, Konno-Rastan procedure provides very successful solutions as an anterior aortoventriculoplasty method. In this article, we performed anterior aortaventriculoplasty surgical treatment for tunnel type left ventricular outflow tract stenosis, recurrent subvalvular discrete membrane, and aortic regurgitation in an adult patient with a history of partial atrioventricular septal defect repair and subvalvular discrete membrane resection operation in early childhood. The Konno-Rastan procedure, which we applied to the redo case, which is rarely used in adult patients and rarely seen in the literature, is shared.     

Isolated pulmonic valve endocarditis in an adult patient with ventricular septal defect and infundibular pulmonary stenosis

Ventricular septal defect associated with infundibular pulmonary stenosis is a relatively uncommon congenital cardiac defect. We report the first case of a patient with perimembranous small ventricular septal defect and infundibular stenosis suffered from pulmonary valve endocarditis and septic pulmonary embolism.     

Giant right coronary artery aneurysm with fistulous connection to the coronary sinus: Diagnosis and management

This report describes the case of a giant aneurysmal right coronary artery connecting to the coronary sinus near its opening into the right atrium in a 70-year-old woman who presented with a recent onset of congestive heart failure. By coincidence, a secundum atrial septal defect was also present. The diagnosis and surgical management of this uncommon pathology are described.     

室间隔缺损合并右室流出道狭窄14例分析

目的总结室间隔缺损合并右室流出道狭窄的临床特点。方法回顾我科2004年6月至2006年10月经手术治疗的室间隔缺损(VSD)合并右室流出道狭窄(RVOTS)14例的临床资料,分析术前漏诊及误诊的原因,探讨VSD RVOTS的病理解剖特征及手术切口的选择。结果本组患者均在浅低温体外循环下行VSD修补、右室流出道疏通术。采用右房切口4例,右房及右室双切口10例。8例VSD用绦纶补片修补,6例直接缝合。4例以心包补片加宽右室流出道。术后未发现低心排及残余分流的病例,2例出现完全型右束支传导阻滞,均痊愈出院。结论VSD RVOTS应与法乐四联症及双腔右心室进行鉴别诊断,造成术前漏诊及误诊是因为对本病认识不足。本组病例均为膜周部中小VSD,RVOTS绝大多数以肌性狭窄为主要改变。RVOTS轻者可经右房切口,但RVOTS严重者宜选择右室流出道纵切口。     

Ventricular Septal Defect Masquerading as Severe Tricuspid Regurgitation: A Case Report and Literature Review

Two-dimensional echocardiography (2-D) is a useful technique for noninvasive evaluation cardiac structure, function, and hemodynamics; however, multiple factors may limit the technical adequacy of the 2-D examination. In this article, we present the case of a ventricular septal defect to right atrial shunt, which was misinterpreted as severe tricuspid regurgitation secondary to severe pulmonary hypertension, despite the absence of right ventricular enlargement or hypertrophy. A transesophageal echocardiography (TEE) examination was performed to explain the discrepancy between the 2-D and Doppler findings, the results of which are discussed, along with a review of the literature.     

右室高位间隔部与心尖部VVI起搏对左心功能及三尖瓣返流影响的比较

目的：探讨右室高位间隔部起搏（HRVS）与右室心尖部起搏（RVA）对左心功能及右心三尖瓣返流的影响。方法：52例接受心室按需型起搏器（VVI）起搏并愿意接受随访的患者,其中接受HRVS起搏27例,接受RVA起搏25例,分别于术前、术后、术后1周、术后12个月、术后24个月在同一起搏频率（657~／min）用超声心动图测量左室射血分数（LVEF）,左室短轴缩短率（LVFS）,三尖瓣返流（TR）和监测血浆脑钠尿肽（BNP）水平。结果：HRVS起搏患者LVEF、LVFS、TR及BNP水平无明显改变,RVA起搏患者LVEF、LVFS降低,TR轻微返流增加,BNP水平升高。结论：HRVS起搏优于RVA起搏,表明右室生理性起搏的重要性。     

Simultaneous device closure of muscular ventricular septal defect and pulmonary valve balloon dilatation.

Percutaneous closure of ventricular septal defect and pulmonary valvuloplasty appears to be an attractive modality when compared to surgical treatment for both lesions. We report successful transcatheter closure of muscular ventricular septal defect with pulmonary valve balloon dilation performed simultaneously in a 27-year-old woman. Technical considerations in such a setting are being discussed.     

Electrophysiologic characteristics of atrial tachycardia originating from the right pulmonary veins or posterior right atrium: double potentials obtained from the posterior wall of the right atrium can be useful to predict foci of atrial tachycardia in right pulmonary veins or posterior right atrium

INTRODUCTION: The right pulmonary veins (RPVs) and posterior wall of the right atrium (PRA) are anatomically located adjacent to each other. The aim of this study was to demonstrate the electrophysiologic characteristics of atrial tachycardia (AT) originating from the PRA or RPVs. METHODS AND RESULTS: A total of 26 consecutive patients with AT originating from the RPVs or PRA underwent detailed atrial endocardial mapping and successful radiofrequency catheter ablation. Eight foci were found in the PRA and 18 foci in the RPVs. Analysis of P wave configuration showed that lead V1 was the most helpful in distinguishing the AT foci between these two sites. In all cases, double potential (DP) configurations were recorded from several electrodes of a multielectrode catheter placed in the PRA, and the first DP component (FP) was the earliest potential recorded from the right atrium during the tachycardia. The amplitude of the FP was higher than that of the second DP component (SP) for AT foci originating in the PRA, whereas the reverse occurred for those in the RPV. The activation sequence of the FP was from superior to inferior for the AT foci in the superior RPV, whereas the reverse occurred for the AT foci in the inferior RPV. CONCLUSION: P wave configuration in lead V1 is helpful in distinguishing AT foci between those originating in the PRA and RPVs. The DPs obtained from the PRA can be useful in predicting whether AT foci originate from the PRA or RPVs.     

Prenatal ultrasound diagnosis of fetal isolated right ventricular noncompaction with pulmonary artery sling: A rare case report

Noncompaction of the ventricular myocardium (NVM), also known as spongy myocardium, is a rare type of cardiomyopathy that has a serious impact on fetuses, children, and adults. NVM mainly affects the left ventricle, as isolated right ventricular noncompaction (IRVNC) is rare. Pulmonary artery sling (PAS) is a rare condition in which the left pulmonary artery anomalously originates from a normal positioned right pulmonary artery, and only a few studies have reported PAS in fetuses. Fetal IRVNC complicated with PAS has not been reported yet. Here, we report a case of IRVNC complicated with PAS that was diagnosed prenatally at 30 weeks gestation and confirmed by postpartum anatomy and pathology.     

Spontaneous embolization of an atrial septal defect occluder device into the left ventricular outflow tract in a patient with pulmonary stenosis

A 24‐year‐old man was admitted to our outpatient clinic for his routine checkup of consecutively percutaneously treated atrial septal defect (ASD) and pulmonary valvular stenosis 45 days ago. A 24 mm ASD occluder device was implanted under transthoracic echocardiographic guidance and 80 mm Hg peak‐to‐peak pulmonary valvular gradient decreased to 20 mm Hg gradient after pulmonary valve dilatation with 23 mm NUMED II transluminal valvuloplasty catheter balloon. Atrial septal defect (ASD) closure is now routinely performed using a percutaneous approach under echocardiographic guidance especially transthoracic echocardiography (TEE). Centrally located, ostium secundum type and less than 3.5 cm in size are considered ideal for device closure. Although there is considerable variation in size and location of the defects, TEE guidance is quite important for this proportion of ASDs. The selection of patients for percutaneous transcatheter closure of a secundum ASD requires accurate information regarding the anatomy of the defect such as its maximal diameter and the amount of circumferential tissue rims.     

An unusual cause of pulmonary vein stenosis: a case report and review of the literature

We describe the case of a patient with the Carney syndrome and several resections for recurrent left atrial myxomas who underwent autotransplantation of the heart with resection of the left and right atria and reconstruction of both atria with bovine pericardium. She subsequently presented with severe shortness of breath, ascites, and peripheral edema. She was found to have stenosis of all four pulmonary veins and severe pulmonary hypertension. We describe the echocardiographic findings and review the literature on assessment of acquired pulmonary vein stenosis.     

Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair

OBJECTIVE—In patients with repaired tetralogy of Fallot, to examine (1) a possible relation between right ventricular enlargement and QRS prolongation, and (2) the effect of right ventricular enlargement caused by pulmonary regurgitation on the right ventricular ejection fraction, evaluated by three dimensional echocardiography, and global function, evaluated by the myocardial performance index.
DESIGN AND PATIENTS—40 patients with repaired tetralogy were studied. Right ventricular volumes were derived from three dimensional echocardiographic data after this method had been validated by comparison with magnetic resonance imaging in 21 patients. Ejection fraction was calculated from end diastolic and end systolic volumes. The Doppler derived myocardial performance index was measured in all patients. Measured data were correlated with QRS duration.
SETTING—Tertiary cardiac centre for congenital heart disease.
RESULTS—There was good agreement between three dimensional echocardiographic and magnetic resonance assessment of right ventricular volumes and ejection fraction. The z score of the right ventricular end diastolic volume and ejection fraction of all patients was 1.35 and −4.15, respectively. Patients with severe pulmonary regurgitation had a lower right ventricular ejection fraction (p < 0.01) and an increased myocardial performance index (p < 0.01) compared with patients with mild to moderate pulmonary regurgitation. The correlation between ejection fraction and right ventricular end diastolic volume was r = −0.35 (p < 0.05). The mean (SD) QRS duration was 131.89 (25.69) ms, range 80-180 ms. The correlation between QRS duration and right ventricular end diastolic volume was r = 0.6 (p < 0.01).
CONCLUSIONS—There is a correlation between the right ventricular size obtained by three dimensional echocardiography and QRS duration on the surface ECG, indicating mechanoelectrical interaction. The severity of pulmonary regurgitation has a negative influence on right ventricular ejection fraction and combined systolic and diastolic global function, as assessed by myocardial performance.


Keywords: tetralogy of Fallot; pulmonary regurgitation; right ventricular enlargement; QRS prolongation     

Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation

Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived RV volumetric indi‐ ces and markers of disease severity (peak oxygen consumption [VO2], NT‐proBNP and atrial and/or ventricular arrhythmias).
Results: Of the 256 patients in the study cohort (age 33 ± 6 years), 187 (73%) had tetralogy of Fallot (TOF) while 69 (27%) had valvular pulmonic stenosis (VPS). TAPSE/ RVSP (r = 0.73, P < .001) and FAC/RVSP (r = 0.78, P < .001) correlated with peak VO2. Among the CMRI‐derived RV volumetric indices analyzed, only right ventricular end‐ systolic volume index correlated with peak VO2 (r = −0.54, P < .001) and NT‐proBNP (r = 0.51, P < .001). These RV‐PA coupling indices were tested in the validation cohort of 218 patients (age 37 ± 9 years). Similar to the study cohort, TAPSE/RVSP (r = 0.59, P < .001) and FAC/RVSP (r = 0.70, P < .001) correlated with peak VO2. TAPSE/RVSP (but not FAC/RVSP) was also associated with arrhythmia occurrence in both the study cohort and validation cohorts.
Conclusion: Noninvasive RV‐PA coupling may provide complementary prognostic data in the management of chronic PR. Further studies are required to explore this clinical tool.