Initial diagnostic errors in children suspected of having heart disease: prevalence and long-term consequences

OBJECTIVE: To access the incidence of diagnostic errors in the initial evaluation of children with cardiac murmurs. METHODS: We evaluated our 7-years of experience in a public pediatric cardiology outpatient clinic. Of 3692 patients who were sent to the hospital, 2603 presented with a heart murmur and were investigated. Patients for whom a disagreement existed between the initial and final diagnoses were divided into the following 2 groups: G1 (n=17) with an initial diagnosis of an innocent murmur and a final diagnosis of cardiopathy, and G2 (n=161) with an initial diagnosis of cardiopathy and a final diagnosis of a normal heart. RESULTS: In G1, the great majority of patients had cardiac defects with mild hemodynamic repercussions, such as small ventricular septal defect and mild pulmonary stenosis. In G2, the great majority of structural defects were interventricular communication, atrial septal defect and pulmonary valve stenosis. CONCLUSION: A global analysis demonstrated that diagnostic error in the initial evaluation of children with cardiac murmurs is real, reaching approximately 6% of cases. The majority of these misdiagnoses were in patients with an initial diagnosis of cardiopathy, which was not confirmed through later complementary examinations. Clinical cardiovascular examination is an excellent resource in the evaluation of children suspected of having cardiopathy. Immediate outpatient discharge of children with an initial diagnosis of an innocent heart murmur seems to be a suitable approach.

Spectrum of cardiac lesions associated with Isolated Cleft Mitral Valve and their impact on therapeutic choices

Abstract Background: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management. Methods: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014. Results: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation. Conclusion: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease

We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P < 0.01), improvement of the Borg dyspnea score from 4.0 (median value) to 3.0 (P < 0.01), and increased pulmonary flow (velocity-time integral) from a median value of 0.12 (range 0.08 to 0.25) to 0.23 (range 0.11 to 0.40; P < 0.01) with no changes in pulmonary pressures. In one patient with pulmonary veno-occlusive disease diagnosed by a lung biopsy, sildenafil had a better effect on the pulmonary wedge pressure than inhaled nitric oxide (15 and 29 mmHg, respectively, acute test). He walked 112 m at baseline and 408 m at one year. One patient died at 11 months of treatment. No other relevant events occurred. Thus, chronic administration of sildenafil improves the physical capacity of PAH patients and may be beneficial in selected cases of veno-occlusive disease.

Ablação por cateter do flutter atrial. Caracterização eletrofisiológica da interrupção da condução pelos istmos posterior e septal

OBJETIVO: Avaliar os tipos de bloqueio obtidos nos istmos posterior (entre o anel tricuspídeo e veia cava inferior) e septal (entre o anel tricuspídeo e óstio do seio coronário), após ablação do flutter atrial (FLA). MÉTODOS: Foram submetidos à ablação por radiofreqüência (RF) 14 pacientes com FLA tipo I (9 homens) em 16 procedimentos. A ativação atrial ao redor do anel tricuspídeo foi avaliada em ritmo sinusal utilizando-se cateter "Halo" com 10 pares de eletrodos (H1-2 a H19-20), durante estimulação do seio coronário proximal (SCP) e região póstero-lateral do átrio direito (H1-2), antes e após ablações lineares. De acordo com a frente de programação do impulso definiu-se: ausência de bloqueio (condução bidirecional), bloqueio incompleto (condução bidirecional com retardo num dos sentidos) e bloqueio completo (ausência de condução pelo istmo). O intervalo desta ativação (deltaSCP/H1-2) foi analisado. RESULTADOS: Bloqueio completo foi obtido em 7 procedimentos (44%) e incompleto em 4 (25%). O deltaSCP/H1-2 foi de 74 ± 26ms no primeiro grupo e de 30,5 ± 7,5ms no segundo (p<0,05). Em 5 casos não demonstrou-se bloqueio. Num seguimento médio de 12 meses, ocorreu recorrência do FLA nos 6 pacientes com bloqueio incompleto ou ausente e em nenhum com bloqueio completo (p<0,001). CONCLUSÃO: A verificação de bloqueio completo pelo mapeamento multipolar atrial é uma estratégia eficaz para definir o sucesso e recorrência clínica na ablação do FLA tipo I.

Aneurysm of the Right Atrial Appendage

Atrial aneurysms involving the free wall or atrial appendage are rare entities in cardiology practice and may be associated with atrial arrhythmias or embolic phenomena. We review the literature and report a case of aneurysm of the right atrial appendage in a young adult, whose diagnosis was established with echocardiography after an episode of paroxysmal atrial flutter.

Vector electrocardiographic alterations after percutaneous septal ablation in obstructive hypertrophic cardiomyopathy: possible anatomic causes

OBJECTIVE: Analyze the dromotropic disturbances (vector-electrocardiographic), and the possible anatomic causes, provoked by selective alcohol injection in the septal branch, for percutaneous treatment, of obstructive hypertrophic cardiomyopathy. METHODS: Ten patients with a mean age of 52.7 years underwent percutaneous septal ablation (PTSA) from october 1998; all in functional class III/IV). Twelve-lead electrocardiogram was performed prior to and during PTSA, and later electrocardiogram and vectorcardiogram according to Frank's method. The patients were followed up for 32 months. RESULTS: On electrocardiogram (ECG) prior to PTSA all patients had sinus rhythm and left atrial enlargement, 8 left ventricular hypertrophy of systolic pattern. On ECG immediately after PTSA, 8 had complete right bundle-branch block; 1 transient total atrioventricular block; 1 alternating transient bundle-branch block either right or hemiblock. On late ECG 8 had complete right bundle-branch block confirmed by vectorcardiogram, type 1 or Grishman. CONCLUSION: Septal fibrosis following alcohol injection caused a predominance of complete right bundle-branch block, different from surgery of myotomy/myectomy.

Um novo índice de doppler tecidual para a predição de fibrilação atrial futura em pacientes com insuficiência cardíaca

FUNDAMENTO: O aparecimento de Fibrilação Atrial (FA) em pacientes com Insuficiência Cardíaca (IC) está em geral associado a uma alta ocorrência de complicações cardiovasculares. Constatou-se que a relação E/(E' × S') (E = velocidade transmitral diastólica inicial, E' = velocidade diastólica inicial no anel mitral e S = velocidade sistólica no anel mitral) reflete a pressão de enchimento do ventrículo esquerdo. Objetivo: Investigamos se E/(E' × S') poderia ser um preditor de FA de início recente em pacientes com IC. MÉTODOS: Foram analisados 113 pacientes consecutivos hospitalizados com IC, em ritmo sinusal, após o tratamento médico adequado. Os pacientes com histórico de FA, imagens ecocardiográficas inadequadas, cardiopatia congênita, ritmo acelerado, doença valvar primária significativa, síndrome coronariana aguda, revascularização coronária durante o seguimento, doença pulmonar ou insuficiência renal grave não foram incluídos. E/(E' × S') foi determinado utilizando a média das velocidades das bordas septal e lateral do anel mitral. A meta principal do estudo foi a FA de início recente. RESULTADOS: Durante o período de seguimento (35,7 ± 11,2 meses), 33 pacientes (29,2%) desenvolveram FA. A média de E/(E' × S') foi de 3,09 ± 1,12 nesses pacientes, ao passo que foi de 1,72 ± 1,34 no restante (p < 0,001). O corte de relação E/(E' × S') ótima para predizer FA de início recente foi de 2,2 (88% de sensibilidade, 77% de especificidade). Havia 64 pacientes (56,6%) com E/(E' × S') < 2,2 e 49 (43,4%) com E/(E '× S') > 2,2. A FA de início recente foi maior em pacientes com E/(E' × S') > 2,2 que em pacientes com E/(E' × S') < 2,2 [29 (59,1%) versus 4 (6,2%), p < 0,001]. Na análise multivariada de Cox incluindo as variáveis que previram FA em análise univariada, a relação E/(E' × S') foi o único preditor independente de FA de início recente (relação de risco = 2,26, 95% de intervalo de confiança = 1,25 - 4,09, p = 0,007). CONCLUSÃO: Em pacientes com IC, a relação E/(E' × S') parece ser um bom preditor de FA de início recente.

Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy

Background:Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA) function in this disease still lacks.Objective:To assess the different LA functions (reservoir, conduit and pump functions) and their correlation with the echocardiographic parameters of left ventricular (LV) systolic and diastolic functions.Methods:10 control subjects (CG), and patients with Chagas disease as follows: 26 with the indeterminate form (GI); 30 with ECG alterations (GII); and 19 with LV dysfunction (GIII). All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging.Results:Reservoir function (Total Emptying Fraction: TEF): (p <0.0001), lower in GIII as compared to CG (p = 0.003), GI (p <0.001) and GII (p <0.001). Conduit function (Passive Emptying Fraction: PEF): (p = 0.004), lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07). Pump function (Active Emptying Fraction: AEF): (p = 0.0001), lower in GIII as compared to CG (p = 0.05), GI (p<0.0001) and GII (p = 0.002). There was a negative correlation of E/e’average with the reservoir and pump functions (TEF and AEF), and a positive correlation of e’average with s’ wave (both septal and lateral walls) and the reservoir, conduit and pump LA functions.Conclusion:An impairment of LA functions in Chagas cardiomyopathy was observed.

HEART ANEURYSM IN CHAGAS' DISEASE

This prospective study on 41 autopsy collected human hearts concerns the "apical" lesion in Chagas' disease. Previous report did not show a correlation between lesion frequency and heart weight then discarding a vascular factor in its pathogenesis. The present paper involves other variables besides the heart weight to evaluate the relative coronary insufficiency. Distinct colored gel (green and red) injected through the capillary beds of both coronary arteries defined the extent of both vessels before separating the atria and removing the sub-epicardium fat. The Right Ventricle (RV) and Left Ventricle (LV) free walls furnished the RV/LV mass ratio. The myocardium mass colored green (right coronary artery - RC) and the whole Ventricular Weight (VW) determined the RC/VW mass ratio. The heart weight plus these mass ratios, graded and added, composed a score inversely proportional to the myocardium irrigation condition. It intended to be a more sensitive morphologic evaluation of the relative ischaemia to correlate to the apical lesion. This study showed a right deviation for the relative accumulated frequency of lesions plotted as a score function and a significant difference for higher scores in hearts with aneurysm. It suggests a ischaemic factor intervening in the apical lesion pathogenesis in Chagas' cardiopathy.

Treatment by splenectomy of a portal vein aneurysm in hepatosplenic schistosomiasis

Portal vein aneurysm is a rare medical entity that can be caused by chronic hepatic diseases with portal hypertension. We describe a 45-year-old man with variceal bleeding from hepatosplenic schistosomiasis and an incidentally found intrahepatic aneurysm. Diagnosis was confirmed with non-invasive imaging exams, arteriography and liver biopsy. Following splenectomy, the aneurysm diameter decreased substantially.

Mycotic aneurysm of the tibioperoneal trunk: a first manifestation of an infected endocarditis

Infrapopliteal mycotic aneurysm resulting from endocarditis is rare, with only a few reported cases. We describe the case of a 28-year-old male patient who was suffering with pain and edema in the right leg. The ultrasound revealed an aneurysm of the right tibioperoneal trunk and a deep vein thrombosis (DVT). The patient was admitted and developed acute congestive heart failure, being diagnosed with possible endocarditis. A pseudo-aneurysm was revealed by arteriography. Aggressive antibiotic treatment was initiated, and open surgery confirmed a mycotic pseudo-aneurysm of the tibioperoneal trunk. To our knowledge, this is the 8th case reported of an infected aneurysm in this particular location.

Pathogenesis of hepatic septal fibrosis associated with Capillaria hepatica infection of rats

Septal fibrosis is a common form of hepatic fibrosis, but its etiology and pathogenesis are poorly understood. Rats infected with the helminth Capillaria hepatica constitute a good experimental model of such fibrosis. To investigate the pathogenetic contribution of the several parasitic factors involved, the following procedures were performed in rats: a) regarding the role of eggs, these were isolated and injected either into the peritoneal cavity or directly into the liver parenchyma; b) for worms alone, 15-day-old infection was treated with mebendazole, killing the parasites before oviposition started; c) for both eggs and worms, rats at the 30th day of infection were treated with either mebendazole or ivermectin. Eggs only originated focal fibrosis from cicatricial granulomas, but no septal fibrosis. Worms alone induced a mild degree of perifocal septal fibrosis. Systematized septal fibrosis of the liver, similar to that observed in the infected controls, occurred only in the rats treated with mebendazole or ivermectin, with dead worms and immature eggs in their livers. Thus, future search for fibrogenic factors associated with C. hepatica infection in rats should consider lesions with both eggs and worms.

Production of septal fibrosis of the liver by means of foreign protein injections into rats

Similarities and differences in antigenic humoral responses and electrophoretic patterns between Capillaria hepatica and pig-serum were investigated as a contribution to the understanding of hepatic fibrosis induced by the parenteral administration of foreign proteins. Only two out of 10 rats receiving repeated intraperitoneal injections of an extract of Capillaria hepatica-infected mouse liver presented septal hepatic fibrosis (20%). Under the same experimental conditions, 4 out of 9 rats (44.4%) developed septal fibrosis following whole pig-serum administration. Injections of normal mouse liver extracts did not result in hepatic fibrosis. Since a 100% septal fibrosis rate is observed in experimentally Capillaria hepatica-infected rats, it appeared that Capillaria hepatica products continuously released from inside the liver creates a much more effective fibrosis inducing mechanism than the parenteral administration of such factors. Thus, repeated peritoneal administration of a foreign protein to rats would not reveal the full fibrogenic potential it may have under natural conditions.