Coronary-cameral fistulas in adults: Acquired types (second of two parts)

Acquired coronary artery fistulas (CCFs) are infrequently detected during conventional coronary angiography. To delineate the characteristics of congenital (first part) and acquired (second part) CCFs in adults, a PubMed search was conducted for papers dealing with congenital or acquired CCFs. None of the publications describing patients with coronary-vascular fistulas were included. Papers dealing with pediatric subjects were excluded. From the world literature, a total of 243 adult patients were selected who had congenital (n = 159/243, 65%) and acquired (n = 84/243, 35%) CCFs. Among the acquired types (n = 72, 85.7%) were traumatic (iatrogenic (n = 65/72, 90%), accidental (n = 7/72, 10%) and (n = 12, 14.3%) spontaneously developing in relation to severe coronary atherosclerosis or myocardial infarction. A high incidence of spontaneous resolution of iatrogenic CCFs resulting from endomyocardial biopsy or following post-septal myectomy was reported. Spontaneous CCFs associated with myocardial ischemia or infarction resolved completely in 8% of the subjects. Early surgical intervention was the treatment of choice in acquired traumatic accidental CCFs. The congenital types are addressed in a previous issue of this journal (first part). In this review (second of two parts, part II), we describe the acquired coronary-cameral fistulas.     

Acquired coronary cameral fistulas: Are these collaterals losing their destination?

BACKGROUND: The majority of coronary cameral fistulas (CCFs) are congenital in origin. On the other hand, acquired coronary cameral fistulas, having various etiopathogenic origins, are increasingly recognized. HYPOTHESIS: The aim of this study was to assess the possible involvement of coronary atherosclerosis in the pathogenesis of acquired coronary cameral fistulas. METHODS: Between 1993 and 1996 coronary cameral fistulas were detected in seven adults patients with coronary atherosclerosis (n = 4) and following myocardial infarction (n = 3) with a mean age of 59.3 years (range 40-77). They were analyzed at our hospital. RESULTS: Myocardial infarction (MI) was documented in six patients and was localized at the same territory of the fistula-related artery in three of them. All patients remained asymptomatic after the detection of the fistula. Five patients had associated cardiac disorders. Two patients were treated conservatively with medical therapy. Coronary artery bypass grafting (CABG) was performed in three patients. One patient died while awaiting CABG. Thirty-four adult cases with acquired CCFs were collected from the current literature. The right coronary artery was the origin of the fistula in 37% and they terminated into the right heart-side in 72% of cases. They remained asymptomatic in 62% of cases. CONCLUSIONS: It could be concluded that acquired CCFs may complicate the course of severe atherosclerosis or myocardial infarction in certain adult patients. The symptomatology and treatment strategy is comparable in the congenital and acquired types. The distribution of involvement of the right or left coronary arteries is equally divided in both the acquired and congenital types. Further studies are needed to investigate the precipitating factors for the occurrence of and incidence of acquired CCFs in patients with severe atherosclerosis or post-MI subjects.     

Current characteristics of congenital coronary artery fistulas in adults: A decade of global experience

AIM:To describe the characteristics of coronary artery fistulas(CAFs) in adults,including donor vessels and whether termination was cameral or vascular. METHODS:A PubMed search was performed for articles between 2000 and 2010 to describe the current characteristics of congenital CAFs in adults.A group of 304 adults was collected.Clinical data,presentations,diagnostic modalities,angiographic fistula findings and treatment strategies were gathered and analyzed.With regard to CAF origin,the subjects were tabulated into unilateral,bilateral or multilateral fistulas and compared.The group was stratified into two major subsets according to the mode of termination;coronary-cameral fistulas(CCFs) and coronary-vascular fistulas(CVFs) . A comparison was made between the two subsets. Fistula-related major complications[aneurysm formation,infective endocarditis(IE) ,myocardial infarction(MI) ,rupture,pericardial effusion(PE) and tamponade] were described.Coronary artery-ventricular multiple micro-fistulas and acquired CAFs were excluded as well as anomalous origin of the coronary arteries from the pulmonary artery(PA) . RESULTS:A total of 304 adult subjects(47%male) with congenital CAFs were included.The mean age was51.4 years(range,18-86 years) ,with 20%older than 65 years of age.Dyspnea(31%) ,chest pain(23%) and angina pectoris(21%) were the prevalent clinical presentations.Continuous cardiac murmur was heard in 82%of the subjects.Of the applied diagnostic modalities,chest X-ray showed an abnormal shadow in 4%of the subjects.The cornerstone in establishing the diagnosis was echocardiography(68%) ,and conventional contrast coronary angiography(97%) .However,multislice detector computed tomography was performed in 16%.The unilateral fistula originated from the left in 69%and from the right coronary artery in 31%of the subjects.Most patients(80%) had unilateral fistulas,18%presented with bilateral fistulas and 2%with multilateral fistulas.Termination into the PA was reported in unilateral(44%) ,bilateral(73%) and multilateral(75%) fistulas.Fistulas with multiple origins(bilateral and multilateral) terminated more frequently into the PA(29%) than into other sites(10.6%)(P=0.000) . Aneurysmal formation was found in 14%of all subjects.Spontaneous rupture,PE and tamponade were reported in 2%of all subjects.In CCFs,the mean age was 46.2 years whereas in CVFs mean age was 55.6 years(P=0.003) .IE(4%) was exclusively associated with CCFs,while MI(2%) was only found in subjects with CVFs.Surgical ligation was frequently chosen for unilateral(57%) ,bilateral(51%) and multilateral fistulas(66%) ,but percutaneous therapeutic embolization(PTE) was increasingly reported(23%,17%and 17%,respectively) . CONCLUSION:Congenital CAFs are currently detected in elderly patients.Bilateral fistulas are more frequently reported and PTE is more frequently applied as a therapeutic strategy in adults.     

Solitary Coronary Artery Fistulas: A Congenital Anomaly in Children and Adults. A Contemporary Review

Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life‐threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in adults and children. From the literature between 1993 and 2004, 236 patients with CAFs were considered for evaluation. The present review is intended to assist cardiologists who are unfamiliar with congenital CAFs in adults by suggesting clues for decision making regarding diagnosis and management. Dyspnea and chest pain represented a frequent 91/128 (71%) clinical symptom in CAFs in adults while in the pediatric age group the majority were silent 105/133 (79%) and dyspnea and chest pain accounted for only 8% of the symptoms. The diagnostic modalities were mainly cardiac catheterization and coronary angiography. On the other hand, in the pediatric patients, echocardiography and coronary angiography mainly guided the diagnosis. Regarding treatment strategy in the reviewed subjects, percutaneous transluminal embolization was performed in 18% of the pediatric and in only 5% of the adult subjects. Surgical ligation (46% vs. 38%) and conservative medical strategies (36% vs. 24%) were reported in both pediatric and adult groups. Presentations of CAFs vary considerably in both groups. These differences include the diagnostic modalities, spontaneous closure, spontaneous rupture, and management. From this review, it seemed that—but it may be biased—surgical ligation remains the major mainstay for closure of CAFs in adult and pediatric populations. Recommendations are necessary for antibiotic prophylaxis and antiplatelet and/or anticoagulant therapy for prevention of endocarditis and thrombotic events in patients with CAFs associated with coronary artery dilatation or aneurysmal formation of the fistulous tract.     

Acquired aortocameral fistula occurring late after infective endocarditis: An emblematic case and review of 38 reported cases

AIM To delineate the features and current therapeutic option of congenital and acquired aortocameral fistulas(ACF) secondary to iatrogenic or infectious disorders.METHODS From a Pub Med search using the term "aortocameral fistula", 30 suitable papers for the current review were retrieved. Reviews, case series and case reports published in English were considered. Abstracts and reports from scientific meetings were not included. A total of 38 reviewed subjects were collected and analyzed. In addition, another case- an adult male who presented with ACF between commissures of the right and noncoronary sinuses and right atrium as a late complication of Staphylococcus aureus infective endocarditis of the AV- is added, the world literature is briefly reviewed.RESULTS A total of thirty-eight subjects producing 39 fistulas were reviewed, analyzed and stratified into either congenital(47%) or acquired(53%) according to their etiology. Of all subjects, 11% were asymptomatic and 89% were symptomatic with dyspnea(21 ×) as the most common presentation. Diagnosis was established by a multidiagnostic approach in 23(60%), single method in 14(37%)(echocardiography in 12 and catheterization in 2), and at autopsy in 2(3%) of the subjects. Treatment options included percutaneous transcatheter closure in 12(30%) with the deployment of the Amplatzer duct or septal occluder and Gianturco coil and surgical correction in 24(63%). CONCLUSION Acquired ACF is an infrequent entity which may occur late after an episode of endocarditis of the native AV. The management of ACF is generally by surgical correction but non-surgical device intervention has recently been introduced as a safe alternative.     

Female Gender and the Risk of Rupture of Congenital Aneurysmal Fistula in Adults

Aims. To delineate the risk factors for rupture of congenital aneurysmal fistulas in adult patients. Methods. We conducted a literature search of the Medline database using Pubmed search interface to identify reports dealing with rupture of congenital aneurysmal fistulas in an adult population. The search included the English and non‐English languages between 1963 and 2005. Results. Fourteen adult patients (12 females) with serious and life‐threatening complications secondary to aneurysmal fistulas were reported. Mean age was 62.9 years. The ethnic origins of these 14 patients were 9 Asian and 5 Caucasian. Most patients have had no other cardiac malformations. Five patients had a history of hypertension. One patient was asymptomatic. In 13 symptomatic patients, the clinical presentation was cardiac tamponade, pericardial effusion, syncope, heart failure, chest pain, dyspnea, fatigue, distal thromboembolic events with infarction, shock, and/or sudden death. Aneurysmal fistulas were identified in 10 patients; of these 6 were of the saccular type. Rupture occurred in 9 patients (8 females and 1 male). Eleven patients were treated surgically with 1 late death. Two male subjects experienced sudden unexpected cardiac death. Conclusion. Rupture of congenital aneurysmal fistulas occurred more often in females. Identified risk factors for rupture, hemopericardium, tamponade, and death were among others saccular aneurysm, Asian ethnic race, origin of the aneurysmal fistulas from the left coronary artery and a history of hypertension may play a role. In this article, we present a literature review of congenital aneurysmal fistulas associated with or without rupture and a case report of a woman with unruptured aneurysmal fistula.     

Coronary arteriovenous fistulas: Collective review and management of six new cases—changing etiology,presentation, and treatment strategy

We considered it worthwhile to review the literature of the last decade (1985–1995) to answer the question whether the etiology and the clinical picture of coronary arteriovenous fistulas (CAVFs) have changed. Furthermore, new therapeutic modalities such as percutaneous transcatheter embolization have been developed. We tried to define the place of these techniques in the therapeutic arena. Clinical presentation and management of six patients with eight congenital CAVFs and 76 subjects with 96 congenital and acquired CAVFs taken from a review of the recent literature are presented. Sixty-three review subjects (78%) were treated medically, with one fatal case. Ligation of the fistula was achieved by surgical techniques in 10% of review subjects, while percutaneous transcatheter embolization (PTE) was performed in nine patients (12%). Percutaneous transcatheter embolization techniques are being increasingly used in the treatment of CAVFs. The etiology of CAVFs has a tendency to show alterations toward the acquired pathogenesis. In 64% of the review subjects the fistula was congenital in origin, and in 36% it had an acquired cause. Among the patients of the current review, the clinical presentations were 55% asymptomatic, 34% chest pain (anginal or atypical), and 13% congestive heart failure. The CAVFs of our six patients are all congenital in origin. In the current review, the clinical presentation showed a trend toward increasing chest complaints (34%) compared with the review (10%) published in the mid 1970s. This may be due to a higher mean age, and hence increased concurrent coronary artery disease due to aging compared with the review population of two decades ago.     

Myocardial ischemia and congestive heart failure from a left main to coronary sinus fistula

A coronary artery fistula (CAF) is a rare congenital anomaly first reported by Krause in 1865. It is defined as a direct communication between the coronary artery and any surrounding cardiac chamber or vascular structure, which bypasses the myocardial capillary bed. The incidence of small CAFs in an adult population, undergoing cardiac catheterization at the Cleveland Clinic, was 0.13%. In the same series, the incidence of large or multiple fistulas was less than that of small fistulas and was present in 0.05% of all patients screened. The natural history of CAF in adults remains undefined, as does the best approach to managing these patients with recommendations for early closure as well as conservative management found in the medical literature. We present a patient initially diagnosed with a clinically silent CAF who presents 10 years later with symptoms and many of the classic findings of a CAF. An alternative model for the management of CAF in adults is discussed.     

Single-center experience with implantable cardioverter-defibrillators in adults with complex congenital heart disease

Adults with congenital heart disease are at risk of lethal ventricular arrhythmias and are candidates for implantable cardiac defibrillator (ICD) therapy, yet implant risks, long-term outcomes, and rates of appropriate and inappropriate ICD therapies are not well characterized. We reviewed clinical, implantation, and follow-up data on all transvenous ICDs in adults with congenital heart disease at the Mayo Clinic from 1991 through 2008. Seventy-three adults with congenital heart disease received 85 ICDs. Implantation diagnoses included tetralogy of Fallot (44%) and congenitally corrected transposition of the great arteries (17%). Implantation indication was occurrence of sustained ventricular arrhythmias (secondary prevention) in 36% and prophylactic (primary prevention) in the remainder. There were no major implant-related complications. During follow-up (2.2 ± 2.8 years, range 0 to 15) 11 patients died and 4 patients received heart or heart/lung transplants. An appropriate shock for a ventricular arrhythmia was observed in 19% of patients and an inappropriate shock was observed in 15% of patients. Likelihood of an appropriate shock was associated with increased subpulmonic ventricular pressure. In conclusion, implantation of transvenous ICDs in adults with congenital heart disease is associated with a low risk of implant complications. In this high-risk adult population the rate of inappropriate ICD shocks is low, whereas the likelihood of appropriate therapy for potentially lethal ventricular arrhythmias is high. These data suggest overall benefit of ICD therapy in adults with congenital heart disease.     

Circadian and seasonal variation of malignant arrhythmias in a pediatric and congenital heart disease population

INTRODUCTION: Recent studies in adult populations have revealed seasonal variation in the frequency of acute cardiovascular events, including life-threatening arrhythmias, demonstrating increased events during winter and early spring. Trends in the time of day that arrhythmias occur also were noted. We sought to establish whether pediatric and young adult congenital heart disease implantable cardioverter defibrillator (ICD) recipients have circadian or seasonal variability in shock frequency, similar to adult populations. METHODS AND RESULTS: Data from ICD patients at six pediatric centers in North America were analyzed to assess the timing of life-threatening arrhythmias. The populations consisted of children and adults with congenital heart disease and ICDs placed for malignant arrhythmias. Data were considered in 46 patients who received appropriate therapy (total 139 episodes) for ventricular tachycardia or ventricular fibrillation. Multiple variables were analyzed, including time of day, day of week, and month of year. In contrast to previously studied adult patients, fewer events occurred in the early morning (7.5%), with the most therapies occurring between 6 P.M. and midnight (35%). An increased frequency of therapies was observed in the fall and winter (September-January), representing 60% of all appropriate shocks. Unlike adult populations, Mondays did not have an increased frequency of malignant arrhythmias. CONCLUSION: Pediatric and adult congenital heart disease populations have moderate seasonal and 24-hour variation in ICD event rate, with some distinctly different peaks than those seen in typical adult ICD populations. These findings suggest circadian variation in arrhythmia vulnerability that may differ from conventional occupational, physical, or emotional stressors. (J Cardiovasc Electrophysiol, Vol. 13, pp.     

Spontaneous acquired left descending coronary artery to left ventricle fistula in a patient with an apical saddle thrombus.

Coronary artery fistulas are a rare anomaly. The majority are congenital in origin, although they may be acquired due to invasive cardiac procedures or trauma. The most common course is between the right coronary artery and the right ventricle. The authors describe the case of an acquired left anterior descending coronary artery to left ventricle fistula, associated with a saddle thrombus, in a patient with a previously normal coronary angiogram and none of the predisposing factors mentioned above. Spontaneous formation of acquired coronary fistulas is reported as a very rare event in the literature.     

永久起搏器和埋藏式心脏复律除颤器（ICD）在遗传性长QT综合征患者中的应用

目的总结合并窦性心动过缓的遗传性长QT综合征（以下简称遗传性LQTS）患者植入永久起搏器和埋藏式心脏复律除颤器（以下简称ICD）的治疗效果,对比分析这两种治疗在预防患者猝死中的差异。方法对我院从2003年6月到2013年6月出院诊断为遗传性LQTS合并窦性心动过缓、植入了永久起搏器或ICD的全部21例患者,结合门诊、电话和程控随访了解患者的生存状况、手术并发症以及晕厥、室性恶性心律失常的发作情况。结果起搏器组男性2例,女性9例,年龄39.3±14.3岁,随访时间50.6±26.3个月,1例患者猝死,2例患者再发晕厥前兆,其中1例最终更换为ICD。ICD组男性2例,女性8例,年龄34.5±11.9岁,随访时间61.4±43.5个月,3例患者接受了ICD的适当治疗,另2例患者接受了ICD的不适当治疗,1例患者术后出现囊袋感染,1例患者更换为永久起搏器。治疗有效率在起搏器组及ICD组分别为72.7%（8/11）和100.0%（10/10）,未达到统计学差异（p=0.21）。不良事件发生率在起搏器组及ICD组分别为27.3%（3/11）和30.0%（3/10）,也未达到统计学差异（p=0.63）。结论对于不能植入ICD的合并窦性心动过缓的遗传性LQTS患者,植入永久起搏器可能是一个较好的替代方法,但对于QTc≥539ms的患者,只有植入ICD才能预防猝死。植入ICD后长期无心脏事件发生的患者,根据患者意愿,可考虑更换为永久起搏器。     

Implantable cardioverter defibrillator lead complications and laser extraction in children and young adults with congenital heart disease: implications for implantation and management

INTRODUCTION: Implantable cardioverter defibrillators (ICDs) are being implanted for primary and secondary prevention of sudden death in children and young adults with congenital heart disease. Over time, ICD leads adhere to venous endothelium and endocardium. Lead removal, when necessary, often requires disruption of this fibrous tissue. METHODS AND RESULTS: We retrospectively reviewed and analyzed our experience with ICD lead extraction in children and young adults with congenital heart disease. From April 1999 through January 2002, 14 patients underwent 15 lead extraction procedures to remove 21 leads (17 ICD leads and 4 pacing or sensing leads). Seven patients had surgically corrected structural heart disease (5 transposition of the great arteries with atrial switch repair and 2 corrected tetralogy of Fallot). Mean patient age at extraction was 17.9 +/- 5.7 years (range 9-32), and mean duration of lead implantation was 42.0 +/- 18.9 months (range 15-75). Fourteen of 15 procedures were performed for lead fracture or failure. A laser sheath was used for 20 of 21 lead extractions. Twenty of 21 leads (95%) were completely extracted. There were three instances of blood loss requiring transfusion. There were no major complications or deaths. CONCLUSION: Young congenital heart disease patients with an ICD are at risk for growth-related lead distortion. The use of a laser sheath is safe and effective for ICD lead extraction in congenital heart disease patients, despite coil adherence and altered anatomy. It may be advisable to avoid dual-coil leads in patients with the potential for future growth.     

Incidental congenital coronary artery vascular fistulas in adults: Evaluation with adenosine-~(13)N-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas(CAFs) using adenosine stress ~(13)N-ammonia positron emission tomography computed tomography(PET-CT).METHODS Congenital CAFs were incidentally detected during coronary angiography(CAG) procedures in 11 adult patients(six males and five females) with a mean age of 64.3 years(range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas(origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress ~(13)N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery(n = 8), right coronary artery(n = 2) and circumflex(n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery(n = 11) or coronary sinus(n = 1). The CAG delineated the characteristics of the fistula(origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas(8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components(23/36, 64%). Adenosine pharmacologic stress ~(13)N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction.CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.     

Utility of routine follow-up defibrillator threshold testing in congenital heart disease and pediatric populations

INTRODUCTION: Recent studies have suggested that routine defibrillation threshold (DFT) testing of implantable cardioverter defibrillators (ICDs) in adults may not be necessary. The congenital heart disease and pediatric populations are a unique group of ICD recipients having a higher incidence of lead failure. We investigated the utility of follow-up DFT testing in this population. METHODS AND RESULTS: The records of 155 ICD recipients at one center were retrospectively reviewed, and patients having one or more follow-up DFT tests were analyzed. The patients were divided into two groups. The "routine" group consisted of 58 follow-up DFT procedures in 46 patients, without known changes in ICD parameters. The "prompted" group consisted of 21 follow-up DFT procedures in 18 patients, motivated by clinical concerns about changes in ICD lead status. Of 58 "routine" DFTs performed at a mean postimplant duration of 32 +/- 23 months (range 2-78), 7 (12%) had reprogramming, and 1 required a hardware change as a result of the testing. Of the 21 "prompted" DFTs performed, 7 required device reprogramming, and 3 required hardware upgrade. Overall, 19 (24%) of 79 procedures detected clinically significant changes, requiring reprogramming or ICD system revision. No complications were seen from follow-up DFT testing. CONCLUSION: A high rate of abnormalities was found at follow-up DFT testing in this population, especially in the group of patients with clinically prompted testing. Clinically indicated DFT testing, as expected, has a high yield of important information on device function in congenital heart disease and pediatric populations.     

Dutch survey of coronary artery fistulas in adults: congenital solitary fistulas

AIMS: Congenital coronary artery fistulas are frequently identified in adult and pediatric populations and they have been associated with various clinical and morphological features. The purpose of this study was to define the clinical and coronary angiographic morphological characteristics of adult patients with congenital solitary CAFs in the Dutch Registry. METHODS AND RESULTS: Fifty-one patients with angiographically documented CAFs were reviewed for clinical evaluation, used non-invasive and invasive diagnostic tools and treatment modalities. Unilateral CAFs were predominant (80%) and 84% of the patients were symptomatic. The most common presenting symptom was angina pectoris (57%). Angina pectoris was present in a quarter of the patients in the absence of coronary artery disease (CAD). Significant CAD was present in 49% of the patients. Twenty-nine percent of the CAFs showed aneurysmal formation, underlying their potential hazard of rupture. Myocardial infarction occurred in 18% of the patients. In 27% of CAFs multiplicity of the origin was found and nearly all fistulas were tortuous (97%). Treatment modalities were conservative medical in 70%, percutaneous transluminal embolisation in 5% and surgical ligation in 25% of the cases. Multiple micro-fistulas from the coronary arteries to the left ventricle were excluded from the study. CONCLUSION: In this national survey series, congenital solitary CAFs can be presented with typical angina pectoris in the absence of obstructive CAD. Fistula-related coronary artery was infrequently involved in the development of ipsilateral myocardial infarction. Tortuousity and multiplicity of the CAFs may determine, for the individual patient, the choice of the currently available treatment modalities.     

Congenital heart disease in pregnancy

Heart disease is a main cause of maternal mortality in the United States and the United Kingdom. Most deaths are from acquired conditions. However, due to the increased survival of children born with congenital heart disease (CHD) over the past 30 years, the population of adults with congenital heart disease in the U.S. now exceeds 1 million. Thus, there are now more adults with CHD than children with CHD. Many of these adult survivors of pediatric heart disease are of childbearing age and are considering pregnancy. This article reviews the literature concerning pregnancy and CHD.     

Single lead catheter of implantable cardioverterdefibrillator with floating atrial sensing dipole implanted via persistent left superior vena cava

Persistent left superior vena cava(LSVC) is a congenital anomaly with 0.3%-1% prevalence in the general population. It is usually asymptomatic but in case of transvenous lead positioning, i.e., for pacemaker or implantable cardioverter defibrillator(ICD), may be a cause for significant complications or unsuccessful implantation. Single lead ICD with atrial sensing dipole(ICD DX) is a safe and functional technology in patients without congenital abnormalities. We provide a review of the literature and a case report of successful implantation of an ICD DX in a patient with LSVC and its efficacy in treating ventricular arrhythmias.     

Spectrum of congenital mitral valve abnormalities associated with solitary undifferentiated papillary muscle in adults

BackgroundCongenital anomaly wherein the mitral valve leaflets are directly attached to the papillary muscle(s) (PM) with or without short under-developed chords is rarely reported in adults. Patients with two PMs with an intervening fibrous bridge have also been included under this head in previous studies.MethodsEchocardiography enables accurate evaluation of the morphology and function of valve leaflets, chordae tendineae, and PM. This report describes a series of six patients aged 56–84 years who had abnormal mitral valve with a large solitary and anomalously inserted PM seen over a period of 3 years. Only those patients who had a single pillar or bridge-like PM and either absent tendinous chords or small under-developed chords were included in the analysis.ResultsAmong 9600 consecutive echocardiograms performed, six patients met the criteria of an abnormal mitral valve with solitary large PM. Two patients underwent mitral valve replacement with partial excision of the PM wherein echocardiographic observations were confirmed. The patients were previously followed with the diagnosis of hypertrophic cardiomyopathy (3) and rheumatic mitral valve disease (3). Multi-planar reconstruction of 3D echocardiographic images provided incremental value in assessing the detailed patho-anatomy of PMs in these cases.ConclusionIn adult patients, a high index of suspicion is required to detect congenital mitral stenosis/regurgitation with large solitary PM (resembling a parachute mitral valve) which may masquerade as hypertrophic cardiomyopathy or rheumatic mitral valve disease.