Evolution of ventricular outpouching through the fetal and postnatal periods: Unabating dilemma of serial observation or surgical correction

Ventricular outpouching is a rare finding in prenatal sonography and the main differential diagnoses are diverticulum, aneurysm, and pseudoaneurysm in addition to congenital cysts and clefts. The various modes of fetal presentation of congenital ventricular outpouching include an abnormal four-chamber view on fetal two-dimensional echocardiogram, fetal arrhythmia, fetal hydrops, and pericardial effusion. Left ventricular aneurysm (LVA)/nonapical diverticula are usually isolated defects. Apical diverticula are always associated with midline thoracoabdominal defects (epigastric pulsating diverticulum or large omphalocele) and other structural malformations of the heart. Most patients with LVA/congenital ventricular diverticulum remain clinically asymptomatic but they can potentially give rise to complications such as ventricular tachyarrhythmias, systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation. The treatment of asymptomatic LVA and isolated congenital ventricular diverticulum is still undefined. In this review, our aim is to outline a systematic approach to a fetus detected with ventricular outpouching. Starting with prevalence and its types, issues in fetal management, natural course and evolution postbirth, and finally the perpetual dilemma of serial observation or surgical correction is discussed.     

Left ventricular pseudoaneurysm complicating a ruptured isolated congenital diverticulum

We report a case of 41-year-old woman who presented with chest tightness and shortness of breath. Transthoracic echocardiogram (TTE) showed left ventricular (LV) pseudoaneurysm of the inferior wall with preserved LV systolic function. Coronary angiogram was normal. Surgical repair of the pseudoaneurysm with a pericardial patch was performed, and pathological results confirmed rupture of an isolated congenital LV diverticulum, as the most likely etiology.     

中国人成人先天性孤立性左室憩室的临床和影像诊断特点

目的　总结成人先天性左室憩室的临床 (和 )影像特点。方法　对 4例左室造影或 (和 )超高速CT(UFCT)、MRI确诊为成人先天性左室憩室患者的临床和影像特点进行分析。结果　临床无症状 ,然而均合并有冠心病 ,经左室造影确诊为先天性孤立性左室肌型憩室 ,其中 2例需UFCT和MRI补充诊断 ;二维超声心动图不仅与左室造影、MRI和UFCT的影像特征一致 ,可弥补左室造影中因左室影像重叠而对憩室观察缺陷的不足 ,而且其独特的多普勒 (Doppler)血流频谱 ,可与真、假室壁瘤鉴别 ,对憩室有确诊价值。结论　 (1)中国成人先天性左室憩室多为孤立性肌型憩室 ;临床多无症状 ,多合并有冠心病 ,甚至心肌梗死 ;左室造影有确诊价值 ,但有时需UFCT或MRI补充。 (2 )超声心动图不仅与MRI和UFCT影像特征一致 ,而且其独特的Doppler血流频谱 ,对左室肌型憩室有确诊价值。     

A Rare Association of Left Ventricular Diverticulum and Noncompaction: Continuum of Common Developmental Abnormality?

A previously healthy 16‐year‐old girl presented with history of fever of 3 months duration without any significant clinical finding on examination. Transthoracic echocardiography (TTE) revealed moderate pericardial effusion and later diagnosed as tubercular in etiology. Besides, TTE also showed an incidental finding of large left ventricular (LV) diverticulum in submitral region along the postero‐basal segment and left ventricular noncompaction (LVNC). Nonapical LV diverticulum is a rare variant of this uncommon congenital condition and its association with LVNC has not been reported in literature. Patient was discharged on antitubercular therapy for pericardial effusion and advised for 6 monthly follow‐up for diverticulum.     

Congenital left ventricular diverticulum diagnosed by 64-detector CT imaging

A congenital left ventricular (LV) diverticulum or aneurysm is a rare cardiac malformation. It is a developmental anomaly that occurs during embryogenesis. Most congenital LV aneurysms and diverticula are asymptomatic or may cause systemic embolization, heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies such as echocardiography, magnetic resonance imaging or left ventriculography, visualizing the structural changes. We report the case of a 28-year-old male referred for the evaluation of atypical chest pain who was found to have an LV diverticulum affecting the inferoposterior wall of his LV.     

Three‐Dimensional Contrast Echocardiography in Assessing Left Ventricular Diverticulum

Congenital left ventricular (LV) diverticulum is a rare malformation that can be alone or in association with other congenital abnormalities. Since the several complications have been reported in association with LV diverticulum, as cardiac rupture, heart failure, and endocarditis, patients with LV diverticulum who are managed with conservative treatment would require regular checkups to ascertain the absence of such complications. We report a case of LV diverticulum in a 47‐year‐old man. He took the serial three‐dimensional contrast echocardiography, which was reliable and readily available tool for defining LV diverticulum and monitoring the presence of complications.     

Diverticular and aneurysmal structures of the left ventricle in adults: report of a case within the context of a literature review

Left ventricular outpouchings are increasingly detected on cardiovascular imaging. Herein, we describe the case of a 45-year-old man who underwent noncardiac preoperative imaging and was found to have an asymptomatic left ventricular outpouching. The patient underwent successful surgical repair of the structure. When left ventricular outpouchings are detected, the main differential diagnoses are pseudoaneurysm, aneurysm, and diverticulum. The outcomes for these conditions differ substantially, and accurate diagnosis can be crucial in making clinical decisions. We review the relevant medical literature, outline the natural history of these left ventricular abnormalities, and discuss options in regard to their management.     

Isolated Congenital Muscular Diverticulum of the Left Ventricular Apex Complicated with Mural Thrombosis in an Adult

We present a case of apical isolated congenital left ventricular (LV) diverticulum complicated with mural thrombosis in an adult and elucidate the echocardiographic features for differential diagnosis of LV outpouchings.     

Successful Aneurysmectomy of a Congenital Apical Left Ventricular Aneurysm

Congenital apical left ventricular aneurysm is a rare clinical entity that is different from congenital left ventricular diverticulum. This aneurysm usually occurs as an isolated anomaly. Its clinical presentation varies, and it is usually diagnosed by exclusion.Herein, we report the case of a 54-year-old man who experienced progressively increasing symptoms of congestive cardiac failure. Through the use of contrast echocardiography and angiocardiography, and upon histopathologic examination, he was diagnosed to have a congenital apical left ventricular aneurysm. He was successfully treated by means of left ventricular aneurysmectomy. We discuss the process of diagnosis and surgical correction of the aneurysm, and we briefly review the pertinent medical literature.Key words: Coronary aneurysm/surgery, diagnosis, differential, echocardiography, heart aneurysm/congenital/diagnosis/radiography/surgery, heart ventricles/abnormalities/radiographyCongenital apical left ventricular (LV) aneurysm, a rare clinical entity that differs from congenital LV diverticulum, usually occurs as an isolated anomaly. The clinical presentation of congenital apical LV aneurysm varies, and the aneurysm is usually diagnosed by exclusion.^1–4 Here, we report the case of a patient who presented with progressively increasing dyspnea. He was diagnosed to have a congenital apical LV aneurysm, for which he underwent aneurysmectomy. The extreme rarity of the disease entity, the use of angiocardiography and contrast echocardiography for diagnosis, the process of surgical correction, and a brief review of the medical literature form the basis of this report.     

Left ventricular diverticulum mimicking ventricular pseudoaneurysm in an adult

True diverticulum of the left ventricle is very rarely seen in adults: the condition typically occurs in children and can be associated with other anatomic defects that involve the thoracoabdominal midline. Left ventricular diverticulum, which is usually asymptomatic and typically discovered incidentally, can pose a substantial challenge to the surgeon.Herein, we report the case of a 46-year-old man who presented with worsening exertional angina and ST-segment elevation in the inferior electrocardiographic leads. After a stent was deployed in the patient's occluded right coronary artery, left ventriculography revealed outward pouching of the left ventricular inferior wall, suggesting an aneurysm or a contained free-wall rupture. Transesophageal echocardiography showed a sizable defect and a possible intracavitary thrombus. The presumptive diagnosis was a postinfarction subacute pseudoaneurysm of the left ventricle. However, during surgery, we saw no clots, intrapericardial blood accumulation, or perforation. A localized area of thinned muscle in the region of the posterior descending coronary artery was consistent with a ventricular diverticulum. The left ventricular epicardial surface was reinforced with a small bovine pericardial patch. The patient's recovery was uneventful. We discuss the forms of congenital left ventricular diverticulum and offer considerations regarding differential diagnosis.     

Left ventricular diverticulum mimicking cardiac tumor

Left ventricular (LV) diverticulum represents a rare and frequently asymptomatic congenital anomaly, which is incidentally discovered during routine transthoracic echocardiography. We present a case of a 66‐year‐old female patient who was admitted to the cardiology department due to incidental finding of a tumor‐like mass associated with the posterior mitral leaflet. Preliminary echocardiographic evaluation revealed a solid structure, suspected of mitral annulus calcification or LV malignancy. However, cardiac contrast‐enhanced computed tomography confirmed the presence of LV diverticulum, partially filled with calcified thrombus. Conservative management was recommended. This case underscores the importance of multimodality imaging for differentiation of LV tumor‐like structures.     

Isolated congenital left ventricular diverticulum in an elderly patient that was identified because of an incidental finding during a complete medical checkup

Congenital left ventricular diverticulum is a rare cardiac malformation in an elderly patient. It frequently is associated with other cardiac or non-cardiac congenital malformations. We present an asymptomatic elderly patient, evaluated because of an incidental finding of a left ventricular anatomic change on chest computed tomography during a complete medical checkup. The diagnosis of isolated congenital left ventricular diverticulum was confirmed by echocardiography and cardiac catheterization. With the general use of a complete medical checkup, the incidental findings of patients with isolated congenital left ventricular diverticulum might increase, which might allow for a valid estimation of the morbidity and mortality of these patients.     

Prenatal Diagnosis of Giant Left Ventricular Diverticulum: Case Report

The distinction between a ventricular aneurysm and diverticulum is difficult. The differences between these two are based on anatomical, histological, and functional criteria. We present a case of prenatal diagnosis of a giant left ventricular outpouching at 28 weeks gestation. During the postnatal period, the neonate underwent surgical correction for the outpouching. Histopathological examination reported the resected segment was a cardiac diverticulum. Our case indicates that differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. Histopathological examinations are sometimes necessary to make a precise differentiation. Treatment should be individualized depending on the clinical presentation.     

Congenital submitral aneurysm and sinus of valsalva aneurysm

Submitral aneurysm (SMA) is congenital outpouching of the left ventricular (LV) wall invariably occurring adjacent to the posterior leaflet of mitral valve. SMA is typically diagnosed in young adults who present with severe mitral regurgitation, heart failure, systemic embolism, and sudden cardiac death. African blacks account for the majority of the reported SMA cases. Our report of the very rare combination of SMA with aortic sinus aneurysm lends support to the congenital origin of this pathology occurring due to developmental deficiency in the fusion of myocardium and cardiac fibro skeleton. Complete diagnosis was made by transthoracic echocardiography.     

Isolated congenital left ventricular diverticulum in adults.

Isolated congenital ventricular diverticulum or aneurysm is rare and usually arises from the left ventricle. The presentation of this condition is diverse. We report three cases of isolated congenital left ventricular diverticula. The age range was 17-30 years. Chest X-ray provided the earliest clinical suspicion in these three cases of a cardiac anomaly which was diagnosed by echocardiography and confirmed by angiocardiography. The location of the congenital left ventricular diverticulum was the left ventricular apex in two cases and basal in the other. We conclude that congenital left ventricular diverticulum is a disease of protean presentations. A high index of suspicion is necessary while interpreting chest X-rays and echocardiographs to diagnose congenital left ventricular diverticulum. A contractile accessory chamber of the left ventricle with a narrow neck with or without midline defects and an electrocardiogram without Q waves is consistent with the diagnosis of congenital left ventricular diverticulum.     

Unusual Cause of Heart Failure in a 65‐Year‐Old Woman

Left ventricular (LV) free wall rupture is a potentially lethal mechanical complication after myocardial infarction (MI). Pericardial adhesions or slow extracardiac leak and pericardial inflammation may result in a contained cardiac rupture. LV pseudoaneurysm is a relatively uncommon clinical entity. It may occur after MI, but also as a complication of infective endocarditis, cardiac surgery, or trauma. Patients developing LV pseudoaneurysm after MI may present angina pectoris or signs of congestive heart failure (HF) but often are asymptomatic. Surgery is the treatment of choice for LV pseudoaneurysms diagnosed in the first months after MI. The management of chronic LV pseudoaneurysms is still subject of debate. This report highlights a 65‐year‐old patient newly hospitalized for acute decompensated HF who was diagnosed with a large chronic LV pseudoaneurysm and severe mitral regurgitation. The patient underwent successful resection of the pseudoaneurysm and patch repair of the ruptured ventricular wall.     

Left ventricular pseudoaneurysm after myocardial infarction

In this report, a case of a left ventricular (LV) pseudoaneurysm due to a previous myocardial infarction, which was repaired successfully, is described. A 62-year-old man, with a history of acute anterior wall myocardial infarction 6 months previously, was admitted with the complaints of acute dyspnea and palpitation. Echocardiography revealed an LV aneurysm, and ventriculography showed ventricular dysfunction and an LV pseudoaneurysm. Coronary angiography showed total occlusion of the proximal segment of the left anterior descending artery with a very thin lumen and insufficient retrograde filling. Under cardiopulmonary bypass and beating heart, the pseudoaneurysm was resected and the defect on the ventricular free wall was closed by the remodeling ventriculoplasty method of Dor. Histopathologic examination of the resected material confirmed the diagnosis of pseudoaneurysm. The postoperative course of our patient was uneventful. He was discharged on the ninth postoperative day.     

Non-invasive assessment of the presence and severity of cardiac amyloidosis. A study in familial amyloidosis with polyneuropathy by cross sectional echocardiography and technetium-99m pyrophosphate scintigraphy.

Of two infants with congenital cardiac diverticula one had a defect consisting of a submitral valvular diverticulum rather than the expected aneurysm. It was clearly shown by echocardiography as well as angiography and was associated with moderate mitral incompetence and a poorly functioning left ventricular wall. The second infant had an isolated apical left ventricular diverticulum with subnormal left ventricular function and severe mitral incompetence. Because of intractable congestive heart failure this infant underwent successful replacement of a dysplastic fibrotic mitral valve which on histological examination had myxomatous features. The association between congenital mitral disease and congenital apical diverticular may be related to race and sex.     

Multiple left ventricular diverticuli in an asymptomatic adult: case report and review of the literature.

Left ventricular (LV) diverticulum is a rare congenital abnormality for which the etiology, management, and natural history are very poorly understood. Although most often observed as a single diverticulum, we report a case of multiple LV diverticuli in an asymptomatic adult referred for coronary evaluation due to an abnormal electrocardiogram. LV diverticuli are often associated with other congenital malformations, but can also be found in isolation. Cardiac magnetic resonance imaging provides enhanced ability to determine size, location, and morphological characteristics, which may improve lesion assessment and management. A review of the condition and reported complications is discussed.