Aortico-left ventricular tunnel with ventricular septal defect: two-dimensional/Doppler echocardiographic diagnosis

Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation and congestive heart failure in childhood. Its association with a ventricular septal defect is even more rare. Because of the distortion of the normal anatomy around the aortic valve and the rarity of this combination of defects, the diagnosis of aortico-left ventricular tunnel with ventricular septal defect may be difficult. The two-dimensional and Doppler echocardiographic findings of aortico-left ventricular tunnel are described.     

Parasystolic ventricular tachycardia with exit block

A rare case of parasystolic ventricular tachycardia with variable exit block concurring with runs of reentrant ventricular tachycardia is described. The reentrant ventricular tachycardia or isolated ventricular extrabeats modified the exit block patterns of the parasystolic tachycardia.     

Isolated ventricular noncompaction

Isolated ventricular noncompaction of myocardium is a rare congenital disease due to an arrest of myocardial morphogenesis during foetal development. It is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. The persistence of myocardial noncompaction is usually an associated anomaly in patients with congenital left or right ventricular outflow tract obstruction. However, isolated noncompaction of myocardium is not associated with any factors that would explain it apart from the foetal arrest of compaction of the ventricular myocardium. The disease results in systolic and diastolic ventricular dysfunction, systemic embolism and ventricular arrhythmias. We describe a case of isolated noncompaction of the ventricular myocardium in a 20-year-old man who presented initially with ventricular tachycardia.     

Isolated right ventricular infarction followed by posterior left ventricular infarction after a few days

This report presents a rare case of isolated right ventricular infarction complicated by bilateral occlusive pulmonary embolism apparently due to right ventricular mural thrombus. Only 2 to 3 weeks later an infarct of the posterior wall of the left ventricle finally occurred. The clinical, pathological and electrocardiographic features of the case are discussed. This case shows that right ventricular infarct can occur without a preceding or simultaneous infarct of the left ventricle.     

Isolated right ventricular infarction.

The literature on isolated right ventricular infarction is reviewed and local experience is reported. Chronic lung disease is an important risk factor. Chest pain and breathlessness are common. Syncope and sudden collapse can also occur. Rhythm disorders include sinus bradycardia, atrial fibrillation and ventricular tachycardia or fibrillation. Atrioventricular block is rare. Hypotension and a right-sided fourth heart sound are common. Cautious use of slow-release nitroglycerin is not hazardous in the absence of hypotension. High doses of steroids and anticoagulants can be helpful. The prognosis is usually good, although sudden collapse can occur due to ventricular fibrillation, rupture of the right ventricular free wall or massive pulmonary embolism.     

Cyanotic patient with right ventricular dilatation and ventricular arrhythmias

Uhl's disease is a very rare congenital anomaly of the heart. Extreme dilatation of the right ventricle is accompanied by virtual absence of the right ventricular myocardium. We report on a 30-year-old woman with ventricular arrhythmias and atrial-septal defect where the diagnosis was made by echocardiography and confirmed by angiocardiography. Diagnostic and therapeutic possibilities are discussed.     

Intermittent ventricular parasystolic bigeminy accompanied by reentrant ventricular extrasystoles.

An extremely rare case of intermittent ventricular parasystolic bigeminy is presented, in which coexisting reentrant ventricular extrasystoles of sinus origin, showing mainly trigeminy, and those of ventricular parasystolic origin, showing a close coupling to the ventricular parasystolic beat, are demonstrated. Such a unique case has never been reported. Timely parasystolic exit block occurring just prior to the anterograde ventricular activation of the sinus impulse was considered to be of critical importance in developing the reentrant extrasystolic trigeminy.     

Left ventricular diverticulum in two adult patients

Left ventricular diverticulum is a rare congenital anomaly. In the adult population, the incidence was reported to be 0.26% in nonselected patients who underwent cardiac catheterization. Diverticula are usually localized near the apex and most often involve the inferior or anterior parietal walls of the left ventricle. In this report, two cases with congenital left ventricular diverticulum are presented, and the pathophysiologic, diagnostic, and therapeutic approaches of this cardiac malformation are discussed.     

Primary ventricular fibrillation complicating acute myocardial infarction

A controversy exists about whether or not primary ventricular fibrillation in patients with acute myocardial infarction is always preceded by premonitory cardiac arrhythmias. The monitoring system in the Toronto General Hospital Coronary Unit yields a permanent record of every heartbeat and provides a unique opportunity to answer this question. Of 851 patients with proved myocardial infarction, primary ventricular fibrillation developed in 20; 12 of these had frequent ventricular arrhythmias before ventricular fibrillation. In 5 patients, ventricular fibrillation occurred without any warning arrhythmia and the remaining 3 patients had only rare ventricular premature beats.     

Closing ventricular septal defects in the cardiac catheterization laboratory

Current results of transcatheter closure of ventricular septal defects using double-umbrella devices are promising. The ventricular septal defect may be congenital muscular, residual after surgical ventricular septal defect repair, or acquired after myocardial infarction. The procedure is successful in 88% of patients, and most patients experience a significant improvement in clinical status. Although adverse events frequently occur at the time of implantation, late events related to the device or procedures are rare.     

Non-compact left ventricular myocardium: current state of the problem

Non-compaction of the left ventricle or spongy myocardium is a rare congenital cardiomyopathy which is characterized by impaired endomyocardial morphogenesis, hypertrophy of left ventricular myocardium with prominent trabeculation, and deep inter-trabecular recesses. According to WHO classification (1995) this disease belongs to unclassified cardiomyopathies. Main clinical signs of ventricular non-compaction are heart failure, ventricular arrhythmias, systemic and pulmonary embolism. Echocardiography which is a basic method of diagnosis can reveal double layer structure of thickened left ventricular wall and multiple prominent trabeculation with wide inter-trabecular spaces. Predominant localizations of pathological process are region of cardiac apex, inferior and lateral left ventricular walls. Treatment of patients with ventricular non-compaction concentrates on elimination of its main clinical symptoms.     

Isolated noncompaction of ventricular myocardium

Isolated noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The diagnosis can be made echocardiographically, and the entity may be associated with problems of cardiac rhythm. We describe two illustrative cases, with Wolf-Parkinson-White syndrome in one, and left bundle branch block in the other. We emphasize that children with problems of rhythm and findings suggestive for left ventricular cardiomyopathy, either hypertrophic or dilated; should be investigated to exclude isolated noncompaction of the ventricular myocardium.     

Syncopal monomorphic ventricular tachycardia with pleomorphism, sensitive to antitachycardia pacing in a patient with Brugada syndrome.

Polymorphic ventricular tachycardia and ventricular fibrillation are the most common arrhythmias in Brugada syndrome, causing syncope or sudden death. Sustained monomorphic ventricular tachycardias are rare in this context. We report the case of a 41-year-old man with repetitive syncopal episodes and an ajmaline-induced characteristic Brugada ECG pattern, in whom episodes of monomorphic ventricular tachycardia with pleomorphism and response to ventricular pacing were documented.     

General concepts and mechanisms of ventricular tachycardia

The predominant mechanism of ventricular tachycardia is thought to be reentry, although this has been proved to exist in definitely a minority of patients studied owing to technical limitations. Other mechanisms, such as spontaneous triggered automaticity, undoubtedly also cause clinical ventricular tachycardia. During electrophysiologic testing, sustained unimorphic ventricular tachycardia can be induced in the majority of patients who have this arrhythmia clinically. It is extremely rare to induce this rhythm in a patient who has not had ventricular tachyarrhythmias. Nonsustained ventricular tachycardia, repetitive ventricular responses, and pleomorphic ventricular tachycardia and ventricular fibrillation are all nonspecific responses to electrical stimulation and thus are not suitable end points for either the baseline study or serial drug testing. Although an optimal pacing protocol for all subjects does not exist, an adequate test should include pacing with multiple drive rates, at least two (and in some cases, three) extrastimuli, and more than one pacing site in the right ventricle. Isoproterenol infusion and left ventricular pacing may also be necessary in selected patients. During serial drug testing, a drug should not be considered effective if more than 15 repetitive beats are induced unless an extremely aggressive pacing protocol is used. Patients treated with drugs predicted to be effective at electrophysiologic study have a better prognosis than those treated with drugs predicted to be ineffective or those treated with drugs selected empirically.     

Transcatheter closure of left ventricular pseudoaneurysm

Left ventricular pseudoaneurysm is a rare complication of myocardial infarction, cardiovascular surgery, trauma, or infection. Untreated left ventricular pseudoaneurysm can have significant morbidity and mortality. Surgical treatment has generally been the standard of care. However, with a sicker and older population, surgical risks can sometimes be significant. We report a case of successful percutaneous closure of left ventricular pseudoaneurysm using coils and a vascular plug. We emphasize the role and importance of multimodality imaging for accurate diagnosis and therapy, and briefly review the literature on the various approaches used for percutaneous closure of left ventricular pseudoaneurysms.     

Papillary fibroelastoma arising from the left ventricular apex associated with nonspecific systemic symptoms

Primary cardiac tumors are very rare findings. Papillary fibroelastomas are benign tumors comprising less than 10% of all primary cardiac neoplasms. They are almost always attached to valve surfaces; their location along the ventricular and atrial walls is exceptional. Affected patients are mostly asymptomatic, however peripheral or central embolic events are not rare. We describe an interesting case of papillary fibroelastoma with nonspecific systemic symptoms, arising directly from the left ventricular apex. So far, only four such cases were reported in current literature.     

Multimodality imaging of a huge subaortic left ventricular aneurysm in a child

A subannular left ventricular aneurysm is very rare, and is mostly considered to be a congenital anomaly. A subannular left ventricular aneurysm is classified based on the type of its own orifice—submitral or subaortic. Subaortic left ventricular aneurysm occurs less frequently compared with a submitral type of subannular aneurysm. We hereby describe a rare case of a huge bilobed subaortic aneurysm, in which the orifice was located just below the left coronary cusp diagnosed with multimodality imaging in a child.     

An unusual case of postoperative giant left ventricular pseudoaneurysm

Pseudoaneurysms of the left ventricle are rare complications of acute myocardial infarction or cardiac surgery. Three years after aneurysmectomy of a true left ventricular aneurysm, a 66 years old man presented with clinical features of congestive heart failure. The echocardiography showed an extra large chamber next to the posterolateral region of the left ventricle with massive thrombus and severe mitral regurgitation. The diagnosis of pseudoaneurysm was made and was subsequently confirmed by radionuclid angiocardiography and surgical findings. Left ventricular pseudoaneurysm formation is a fairly unusual and serious complication of left ventricular aneurysmectomy with potential catastrophic results. Within the long period of time the pseudoaneurysm insidiously may become giant and may result in heart failure. Recognition of this rare complication of aneurysmectomy has therapeutic importance because surgical correction is necessary. Such pseudoaneurysm formation is easily recognized by two-dimentional echocardiography and radionuclic angiocardiogram. Careful echocardiographic examination is warranted for detecting such a complication in patients after cardiac surgery.     

Acute coronary artery occlusion and ischemia-related ventricular tachycardia during catheter ablation in the right ventricular outflow tract

Coronary artery injury is a rare complication of catheter ablation in the right ventricular outflow tract (RVOT). Furthermore, acute myocardial ischemia usually causes polymorphic ventricular tachycardia (VT) or ventricular fibrillation. We herein describe a case in which catheter ablation for VT originating from the RVOT provoked ischemia-related VTs due to acute occlusion of the left anterior descending artery.     

Right ventricular dysfunction in chronic lung disease

Right ventricular (RV) dysfunction arises in chronic lung disease when chronic hypoxemia and disruption of pulmonary vascular beds increase ventricular afterload. RV dysfunction is defined by hypertrophy with preserved myocardial contractility and cardiac output. RV hypertrophy seems to be a common complication of chronic and advanced lung disease. RV failure is rare, except during acute exacerbations of chronic lung disease or when multiple comorbidities are present. Treatment is targeted at correcting hypoxia and improving pulmonary gas exchange and mechanics. There are no data supporting the use of pulmonary hypertension-specific therapies for patients with RV dysfunction secondary to chronic lung disease.