Percutaneous closure of ductus arteriosus with the amplatzer prosthesis. The Brazilian experience

OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0±1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4±3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

Intrauterine ductus arteriosus constriction: analysis of a historic cohort of 20 cases

OBJECTIVE: To describe the relative incidence, presentation, and evolvement of fetuses with early ductus constriction. METHODS: Twenty fetal echocardiograms indicating ductus constriction were reviewed in a population of 7000 pregnants. RESULTS: The cases were divided into group A (related to maternal use of cyclooxygenase inhibitors [n=7] and group B (idiopathics [n=13]). Mean gestational age was 32.5±3.1 (27-38) weeks and maternal age was 28.2±8.5 (17-42) years. Mean systolic velocity in the ductus was 2.22±0.34 (1.66-2.81) m/s, diastolic velocity 0.79±0.28 (0.45-1.5) m/s, and pulsatility index 1.33±0.36 (0.52-1.83). Two cases of ductal occlusion were noted. In 65% of the cases, an increase occurred in the right cavities; in 90% of the cases, tricuspid or pulmonary regurgitation, or both, occurred, with functional pulmonary atresia in 1 case. Diastolic velocity was greater in group A (1.13±0.33) than in group B (0.68±0.15) (P=0.008). The other data were similar in the 2 groups. The evolvement was not favorable in 4 patients from group B, including 1 death and 2 cases of persistent pulmonary hypertension. CONCLUSION: The high incidence of idiopathic constriction of the ductus arteriosus suggests that its diagnosis is underestimated and that many cases of persistence of fetal circulation in newborns may be related to constriction of the ductus arteriosus not diagnosed during intrauterine life. Group B had a lower severity but a risk of an unfavorable evolvement, suggesting a distinct alteration.

Silent Patent Ductus Arteriosus Aneurysm

Ductus arteriosus aneurysm, a rare and potentially fatal condition, has been reported as a complication after surgical ductus arteriosus closure. Its spontaneous appearance as a septic complication, which was common in the presurgical and preantibiotic era, has been rarely reported in the contemporary literature. Persistence of silent ductus arteriosus in healthy children and adults is a frequent condition that currently has an increasing diagnostic possibility due to the availability of more accurate investigative methods, especially echocardiography. We report the case of a 1-year-old child, in whom no previous heart disease was known, who developed a giant aneurysm of the ductus arteriosus during a staphylococcal infection. This complication appeared after craniotomy for emptying an accidental subdural hematoma. This report associates the persistence of ductus arteriosus with a complication considered rare, which has a rapidly fatal evolution.

Endomyocardial fibrosis in infancy

The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age.

Aortopulmonary window. Clinical and surgical assessment of 18 cases

OBJECTIVE: Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS: Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS: Heart failure occurred in 14 patients, and cyanosis in 3: 2 from GB (tetralogy of Fallot - TF, and double outlet right ventricle - DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION: APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.

Association of pulmonary atresia with intact ventricular septum and aortic valve stenosis. Prenatal diagnosis

A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

A New Use for the Amplatzer Duct Occluder Device

We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel.

Características morfológicas angiográficas na atresia pulmonar com septo interventricular íntegro

OBJETIVO: Determinar as alterações morfofuncionais do ventrículo direito na atresia pulmonar com septo íntegro (APSI) para uma avaliação de candidatos aos diversos procedimentos terapêuticos atualmente disponíveis. MÉTODOS: Submetidos ao estudo cineangiocardiográfico utilizando-se projeções axiais, 31 pacientes com idades variando de 1 a 50 dias (x=9,6), sendo que 28 foram estudados no 1º mês de vida. Na análise estatística foram empregados o X² e calculado o intervalo de confiança de 95% (IC95), o teste de Kruskal-Wallis para a média e desvio padrão e a regressão múltipla. Considerado significativo quando alfa < 0,05. RESULTADOS: Os pacientes foram divididos em 3 grupos de acordo com a morfologia angiográfica do ventrículo direito (VD): grupo A - VD tripartide (n=16); grupo B - VD bipartide (n=9) e grupo C - VD unipartide (n=6). O diâmetro da válvula tricúspide foi de 10,28 ± 2,67 mm (A); 7,82 ± 3,41 (B) e 5,27 ± 0,57 (C) (p=0,0005). A atresia pulmonar foi da válvula em todos do grupo A e infundibular em todos do grupo C (p<0,0001). As conexões coronário-cavitárias foram infreqüentes (2/16) no grupo A e em todos do grupo C (p=0,0006), com opacificação retrógrada da aorta (fluxo do VD para a aorta) em 2 pacientes do grupo A e em todos os do grupo C (p=0,0003). Em 3 pacientes (2 do grupo C e 1 do grupo A) observou-se circulação coronariana VD dependente. A regurgitação tricúspide moderada/grave isolada teve tendência de ser mais freqüente no grupo A (p=0,0525). O ângulo que o ductus arteriosus faz com a aorta descendente foi: 104,06 ± 8,98 no grupo A; 79,17 ± 33,08 no grupo B e 39,0 ± 6,52 no grupo C (p=0,0016). A correlação entre o diâmetro da válvula tricúspide e o ângulo entre o ductus arteriosus com a aorta descendente foi 0,6568 (p=0,0002). CONCLUSÃO: Em função da heterogeneidade da morfologia do VD nos pacientes com atresia pulmonar com septo íntegro, torna-se necessário o conhecimento de todas essas informações na seleção de candidatos aos diversos procedimentos terapêuticos.

Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar

OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1) veia pulmonar encunhada, 2) colaterais aortopulmonares, 3) aorta torácica e 4) ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.

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.

Maternal neurotic symptoms and infants' risk of developing persistent diarrhoea

A previously calculated predictive model for health risk selects infants who suffer 4-5 times more morbidity than their unselected peers. Preliminary results suggested that this risk is related to maternal neurotic symptomatology. To evaluate this hypothesis, 52 consecutive mothers whose infants had a positive predictive score (Group 1) and 52 in whom this was negative (Group 2) were evaluated by means of Goldberg's General Health Questionnaire (GHQ - 30). A total of 41.9% and 20.5% of the mothers in Groups 1 and 2, respectively, scored above 11 points in GHQ-30, established as the cut off point. It is concluded that among poor urban families in Santiago mothers of infants with high risk of persistent diarrhoea have increased frequency of detectable neurotic symptoms. New programs aimed at this type of infant should include psychological support for their mothers.

Outbreak of persistent cutaneous abscesses due to Mycobacterium chelonae after mesotherapy sessions, Lima, Peru

Outbreaks of rapidly growing mycobacteria have been occasionally described. The article reports an outbreak of cutaneous abscesses due to Mycobacterium chelonae following mesotherapy in Lima, Peru. From December 2004 through January 2005, 35 subjects who had participated in mesotherapy training sessions presented with persistent cutaneous abscesses. Thirteen (37%) of these suspected cases consented to underwent clinical examination. Skin punch-biopsies were collected from suspicious lesions and substances injected during mesotherapy were analyzed. Suspected cases were mainly young women and lesions included subcutaneous nodules, abscesses and ulcers. Mycobacterium chelonae was isolated from four patients and from a procaine vial. In conclusion, it is important to consider mesotherapy as a potential source of rapidly growing mycobacteria infections.

Serological diagnosis of toxoplasmosis: usefulness of IgA detection and IgG avidity determination in a patient with a persistent IgM antibody response to Toxoplasma gondii

We report the detection of specific IgA antibodies and the determination of IgG avidity in sequential serum samples from a patient exhibiting significant levels of Toxoplasma-specific IgM antibodies for seven years after the onset of the clinical symptoms of toxoplasmosis. IgM antibodies were detected by an indirect immunofluorescence test and by three commercial enzyme-linked immunosorbent assays (ELISA). Anti-T. gondii IgA was quantified by the a-capture ELISA technique using a commercial kit. As defined by the manufacturer of the IgA ELISA test used, most patients with acute toxoplasmosis have antibody levels > 40 arbitrary units per ml (AU/mL). At this cut-off level, the patient still had a positive ELISA result (45 AU/mL) in a serum sample taken one year after the beginning of clinical manifestations. The IgG avidity-ELISA test was performed with the Falcon assay screening test (F.A.S.T.®) - ELISA system. Avidity indices compatible with a recent Toxoplasma infection were found only in serum samples taken during the first 5 months after the onset of the clinical symptoms of toxoplasmosis. These results show that the interpretation of positive IgM results as indicative of recently acquired toxoplasmosis requires additional laboratory confirmation either by other tests or by the demonstration of a significant rise in the antibody titers in sequential serum samples.

Persistent infections in chronic Chagas' disease patients treated with anti-Trypanosoma cruzi nitroderivatives

We used a molecular method and demonstrated that treatment of the chronic human Trypanosoma cruzi infections with nitroderivatives did not lead to parasitological cure. Seventeen treated and 17 untreated chronic Chagas' disease patients, with at least two out of three positive serologic assays for the infection, and 17 control subjects formed the study groups. PCR assays with nested sets of T. cruzi DNA primers monitored the efficacy of treatment. The amplification products were hybridized to their complementary internal sequences. Untreated and treated Chagas' disease patients yielded PCR amplification products with T. cruzi nuclear DNA primers. Competitive PCR was conducted to determine the quantity of parasites in the blood and revealed < 1 to 75 T. cruzi/ml in untreated (means 25.83 ± 26.32) and < 1 to 36 T. cruzi/ml in treated (means 6.45 ± 9.28) Chagas' disease patients. The difference between the means was not statistically significant. These findings reveal a need for precise definition of the role of treatment of chronic Chagas' disease patients with nitrofuran and nitroimidazole compounds.

Inhaled nitric oxide in the management of persistent pulmonary hypertension of the newborn: a meta-analysis

OBJECTIVES: To evaluate the use of inhaled nitric oxide (NO) in the management of persistent pulmonary hypertension of the newborn. METHODS: Computerized bibliographic search on MEDLINE, CURRENT CONTENTS and LILACS covering the period from January 1990 to March 1998; review of references of all papers found on the subject. Only randomized clinical trials evaluating nitric oxide and conventional treatment were included. OUTCOMES STUDIED: death, requirement for extracorporeal membrane oxygenation (ECMO), systemic oxygenation, complications at the central nervous system and development of chronic pulmonary disease. The methodologic quality of the studies was evaluated by a quality score system, on a scale of 13 points. RESULTS: For infants without congenital diaphragmatic hernia, inhaled NO did not change mortality (typical odds ratio: 1.04; 95% CI: 0.6 to 1.8); the need for ECMO was reduced (relative risk: 0.73; 95% CI: 0.60 to 0.90), and the oxygenation was improved (PaO2 by a mean of 53.3 mm Hg; 95% CI: 44.8 to 61.4; oxygenation index by a mean of -12.2; 95% CI: -14.1 to -9.9). For infants with congenital diaphragmatic hernia, mortality, requirement for ECMO, and oxygenation were not changed. For all infants, central nervous system complications and incidence of chronic pulmonary disease did not change. CONCLUSIONS: Inhaled NO improves oxygenation and reduces requirement for ECMO only in newborns with persistent pulmonary hypertension who do not have diaphragmatic hernia. The risk of complications of the central nervous system and chronic pulmonary disease were not affected by inhaled NO.