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In a previous cross-sectional screening study of 5,169 middle and high school students (mean age, 13.1 ± 1.78 yr) in which we estimated the prevalence of high-risk cardiovascular conditions associated with sudden cardiac death, we incidentally detected by cardiac magnetic resonance (CMR) 959 cases (18.6%) of left ventricular noncompaction (LVNC) that met the Petersen diagnostic criterion (noncompaction:compaction ratio >2.3). Short-axis CMR images were available for 511 of these cases (the Short-Axis Study Set). To determine how many of those cases were truly abnormal, we analyzed the short-axis images in terms of LV structural and functional variables and applied 3 published diagnostic criteria besides the Petersen criterion to our findings. The estimated prevalences were 17.5% based on trabeculated LV mass (Jacquier criterion), 7.4% based on trabeculated LV volume (Choi criterion), and 1.3% based on trabeculated LV mass and distribution (Grothoff criterion). Absent longitudinal clinical outcomes data or accepted diagnostic standards, our analysis of the screening data from the Short-Axis Study Set did not definitively differentiate normal from pathologic cases. However, it does suggest that many of the cases might be normal anatomic variants. It also suggests that cases marked by pathologically excessive LV trabeculation, even if asymptomatic, might involve unsustainable physiologic disadvantages that increase the risk of LV dysfunction, pathologic remodeling, arrhythmias, or mural thrombi. These disadvantages may escape detection, particularly in children developing from prepubescence through adolescence. Longitudinal follow-up of suspected LVNC cases to ascertain their natural history and clinical outcome is warranted.

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Aorto-left ventricular tunnel is a rare congenital cardiac anomaly, consisting of a short abnormal pathway, usually from a sinus of Valsalva into the left ventricular cavity. It is usually diagnosed with echocardiography. We report a case of a newborn presenting with heart murmur and rapid progression to heart failure and left ventricular enlargement due to an aorto-left ventricular tunnel. Despite successful closure of the tunnel, the patient required a Ross procedure due to progressive aortic disease.

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Isolated ventricular noncompaction, a rare genetic cardiomyopathy, is thought to be caused by the arrest of normal myocardial morphogenesis. It is characterized by prominent, excessive trabeculation in a ventricular wall segment and deep intertrabecular recesses perfused from the ventricular cavity. The condition can present with heart failure, systematic embolic events, and ventricular arrhythmias. Two-dimensional echocardiography is the typical diagnostic method. We report a case of heart failure in a 35-year-old man who presented with palpitations. Two-dimensional echocardiograms revealed left ventricular noncompaction, which markedly improved after standard heart failure therapy.

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Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction. Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient's apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.

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BACKGROUND: Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry. METHODS: All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase. RESULTS: In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p<0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation. CONCLUSION: It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population.

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BACKGROUND: There are no clear guidelines for the use of angiotensin-converting enzyme (ACE) inhibitors in patients with single ventricle physiology, especially after the Fontan procedure. Despite this, ACE inhibitors are widely prescribed within the single ventricle population. OBJECTIVE: We decided to review the literature to better establish the efficacy of ACE inhibitors in the single ventricle population and summarise their indications. METHODS: A review of the literature was conducted to identify relevant articles describing use of ACE inhibitors in single ventricle patients both before and after the Fontan procedure. RESULTS: A search in the Ovid MEDLINE and Embase databases identified a total of 140 articles, of which 31 were deemed relevant to the review. CONCLUSIONS: There is little published evidence supporting the use of ACE inhibitors in patients with single ventricle physiology. Based on the current literature, it is possible that ACE inhibitors are overprescribed in patients with single ventricle physiology. The coordination of large, prospective studies through initiatives such as the Australia and New Zealand Fontan Registry is necessary to guide the appropriate use of ACE inhibitors in the single ventricle population.

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Neisseria elongata, a common oral bacterium, has been recognized as a cause of infections such as infective endocarditis, septicemia, and osteomyelitis. Neisseria-induced infective endocarditis, although infrequently reported, typically arises after dental procedures. Without antibiotic therapy, its complications can be severe. We report the case of a 27-year-old man who presented with fever, severe dyspnea, and a leg abscess from cellulitis. An echocardiogram showed a vegetation-like echogenic structure on the septal leaflet of the patient's native tricuspid valve, and an insignificant Gerbode defect. Three blood cultures grew gram-negative, antibiotic-susceptible coccobacilli that were confirmed to be N. elongata. Subsequent DNA sequencing conclusively isolated N. elongata subsp nitroreducens as the organism responsible for the infective endocarditis. The patient recovered after 21 days of antibiotic therapy. In addition to the patient's unusual case, we discuss the nature and isolation of N. elongata and its subspecies.

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Noncompaction of the ventricular myocardium is a congenital cardiomyopathy characterized by prominent ventricular trabeculations and deep intertrabecular recesses. In most cases, noncompaction is an isolated disease confined to the left ventricular myocardium. Fertile eunuch syndrome is a hypogonadotropic hormonal disorder in which the levels of testosterone and follicle-stimulating hormone are low. We report a case of biventricular noncompaction in association with bicuspid aortic valve and severe aortic stenosis in a 42-year-old man who was diagnosed with talipes equinovarus and fertile eunuch syndrome during childhood.

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Aorto-left ventricular tunnel, characterized by extracardiac communication between the ascending aorta and left ventricle, is a very rare congenital condition. Although some affected infants remain asymptomatic until adulthood, most present with symptoms of heart failure during their first year of life. We report the case of an 11-year-old boy who had coexisting abnormalities: an aorto-left ventricular tunnel originating from the left coronary sinus, and an ascending aortic aneurysm. The patient underwent on-pump surgical correction and was in New York Heart Association functional class I status a year later. We also review the nature of this rare condition.

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The major clinical features of myocardial noncompaction are heart failure, arrhythmias, and thromboembolic events. Prominent myocardial trabeculae and deep recesses characteristic of myocardial noncompaction can cause stagnant blood flow and the formation of left ventricular clots. We describe the case of a 62-year-old woman who presented with symptoms of heart failure secondary to left ventricular noncompaction. Transthoracic and transesophageal echocardiography revealed multiple left ventricular thrombi, which had formed despite the patient's long-term therapy with aspirin. Anticoagulative therapy should be considered for patients with myocardial noncompaction who also have risk factors for thromboembolism, such as atrial fibrillation, a history of systemic embolism, or severe left ventricular systolic dysfunction. However, chronic antiplatelet therapy may not sufficiently prevent clot formation in patients who have myocardial noncompaction and severe left ventricular systolic dysfunction.

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Isolated ventricular myocardial noncompaction is a cardiomyopathy that is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. It is an extremely rare cause of heart failure in nonagenarians. We describe the case of a man who presented with heart failure for the first time at 90 years of age. The diagnosis was isolated left ventricular noncompaction. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the left ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 3-month follow-up examination. Knowledge of ventricular noncompaction is increasing within the cardiology community. Patients who have isolated noncompaction with a limited number of involved ventricular segments can live beyond normal life expectancy without developing heart failure. In addition to discussing our patient's case, we briefly review the relevant medical literature.

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Double-chambered right ventricle is a congenital anomaly in which the right ventricle is divided into 2 portions by anomalous muscle bundles. These cases often present in children, but rarely in adults. We discuss 2 cases of double-chambered right ventricle, in patients aged 42 and 35 years. When cases remain asymptomatic until adulthood, they can present with unusual symptoms that lead to incorrect diagnosis. Our cases represent 2 very different manifestations of double-chambered right ventricle, which differ in presentation, in the site of abnormal obstructive muscle bundles, and in the presence of associated lesions. Both of our patients underwent successful surgical resection of the obstruction. One patient also underwent closure of a ventricular septal defect. We also review the literature on the various mechanisms that have been proposed to account for the complex morphology of the abnormal muscle bundles.

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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.

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We describe a case of superoinferior ventricles with an unusual superior location of the left ventricle and inferior location of the right ventricle. The patient was a 17-year-old boy who had been admitted for paroxysmal supraventricular tachycardia with pre-excitation syndrome. The cardiac anomaly was first recognized after admission, and the pre-excitation syndrome, eventually, was treated successfully by radiofrequency catheter ablation. This case featured a segmental set notation {S, D, S} with concordant atrioventricular and ventriculoarterial alignments. Persistent left superior vena cava was the only associated defect. To the best of our knowledge, there has been only 1 other reported case of superoinferior ventricles in which the left ventricle was in the superior position. In our case, the patient's hemodynamics were sufficiently normal that he had reached adolescence with only minor adverse events.

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Biventricular repair is usually difficult to achieve in patients who have right atrial isomerism, which is typically associated with other complex cardiac anomalies. The procedure can be used in patients who have balanced ventricular structures. Herein, we report a successful surgical reconstruction, including biventricular repair, in a 4-year-old boy. The child's right atrial isomerism was associated with double-outlet right ventricle, a large atrial septal defect, a subaortic ventricular septal defect, valvular and infundibular pulmonary stenosis, left persistent superior vena cava, and hemiazygos continuation of an interrupted inferior vena cava. Balanced ventricles enabled biventricular repair, which we consider to be preferable to the Fontan procedure in such circumstances.

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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.

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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.

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Left ventricular noncompaction is a congenital cardiomyopathy, which is often first diagnosed in adults. The condition can be found in isolation, but it has also been described in association with other cardiac anomalies. We report here the 4th documented case of left ventricular noncompaction associated with a bicuspid aortic valve and the 1st of these cases in which the patient underwent aortic valve surgery.

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Herein, we describe the case of a 50-year-old man who had a fistula, located between the left anterior descending coronary artery and the left ventricle, that caused myocardial infarction. Electrocardiography revealed a loss of R-wave progression in leads V(1) through V(4), and transthoracic echocardiography showed an apical aneurysm. Selective coronary angiography was performed. Dimensions of the left anterior descending coronary artery, and digital caliper measurements of stenosis within, were normal. After the injection of angiographic contrast material from the distal part of the left anterior descending coronary artery, a smoky, opaque accumulation colored the left ventricle. The digital caliper measurement of the left anterior descending coronary artery was again found to be normal. In the apex of the left ventricle, ventriculography in the left anterior oblique position revealed a small aneurysm, and a myocardial perfusion scan showed a fixed perfusion defect.Cases of coronary fistulae that result in myocardial infarction are rare. Because the patient had no ongoing symptoms after his myocardial infarction, medical therapy was prescribed.

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