Fallot tetralogy, absent pulmonary valve. main pulmonary arteries. Usually, this anomaly accompanies tetralogy of Fallot with annular pulmonary stenosis and interventricular septal defect. Differential diagnosisOther heart diseases associated with pulmonary valve regurgitation may also have manifestations similar to those previously described. Among them, we can mention pulmonary valve stenosis, heart diseases with pulmonary artery dilatation due to increased pulmonary blood flow, and also heart diseases accompanied by pulmonary arterial hypertension, all in the long term. Diagnostic confirmationThe clinical findings were decisive for the diagnosis of tetralogy of Fallot with absent pulmonary valve, particularly the systolic-diastolic murmur with mild hypoxemia (bidirectional shunt through the VSD) and, above all, the characteristic radiographic image.Echocardiography confirmed this diagnosis, showing significantly dilated pulmonary arteries with diameters of 24 mm on the right and 14 mm on the left. The pulmonary annulus was narrow, measuring 8 mm; there was also infundibular pulmonary stenosis with anterior deviation of the infundibular septum and a large subaortic VSD, with 15 mm of diameter. Pulmonary valve regurgitation and tricuspid valve regurgitation were also present. The ventricles were dilated (Figure 2).Cardiac catheterization showed similar pressures in both ventricles (80/10 mmHg), and the pressure in the main pulmonary artery (30/5 mmHg) evidenced severe pulmonary valve regurgitation, in addition to pulmonary valve stenosis. ManagementDuring surgery, ECC time was 160 min and anoxia time was 70 min. The infundibular stenosis was resected via the right atrium; the absent pulmonary valve was visualized via the pulmonary trunk, as remaining thick and retracted buds in a narrow pulmonary ring. The RV outflow tract was enlarged to the pulmonary trunk and the VSD was closed with bovine pericardium. Number-18 decellularized homograft was implanted in the pulmonary position, and the tricuspid regurgitation was repaired using annuloplasty.The patient had a favorable outcome and was discharged seven days later. Clinical dataThe patient presented with hypoxia in the neonatal period, and for this reason an early systemic-to-pulmonary shunt was indicated at 13 days of life. Shortness of breath since birth did not prevent her from performing full physical activity. She continued to present with mild cyanosis on exertion, although the Blalock-Taussig shunt had not been patent since she was one year old. The patient was receiving digoxin sinceas of three years of age.On physical examination, she was in good general state of health, with no respiratory distress or cyanosis and her pulses were normal. Wake arterial oxygen saturation was 88.0% and the aorta was not palpable at the suprasternal notch.The cardiovascular examination revealed slightly forceful precordial impulses at the left sternal border, and the apex impulse was palpable at the 4th left intercostal space. The heart sounds were accentuated, and a grade...
Congenital cardiopathies; ventricular septal defect, aortic coarctation, pulmonary artery /abnormalities. trunk and the pulmonary arteries demonstrates the presence of pulmonary flow directly from the left ventricle to the right ventricular outflow tract, as in the subpulmonary location of the ventricular septal defect. Differential diagnosisAll other acyanotic congenital cardiopathies with left-toright shunt must be recalled, such as interatrial communication and patent ductus arteriosus, as long as they present a marked hemodynamic effect, which occurs, however, at more advanced ages. This alteration of marked dilatation of the pulmonary trunk is also seen in cyanotic cardiopathies with pulmonary hyperflow, such as total anomalous pulmonary venous connection. Diagnostic confirmationThe clinical elements were decisive for the diagnosis of the ventricular septal defect and aortic coarctation. The echocardiogram confirmed the presence of a ventricular defect with a direct connection with the pulmonary valve in a subpulmonary location, measuring 10 mm in diameter, in addition to the aortic coarctation in the isthmus region, measuring 3 mm in diameter, in relation to 7 mm of the ascending aorta and 5 mm of the aortic arch. The right ventricle was not dilated, considering the preferential flow from the left ventricle to the pulmonary artery tree. The left ventricle was enlarged and the left ventricular function was preserved (LVEF: 68%, Ao: 14, LA: 18, LV: 27) (Fig.2). ManagementAt surgery, the resection of the aortic coarctation in terminoterminal anastomosis with the aortic arch resulted in an adequate diameter of the region. The 10-mm subpulmonary or committed ventricular septal defect was closed through an incision in the pulmonary trunk, using a bovine pericardial patch. The heart failure condition was resolved. CommentsThe marked dilatation of the pulmonary trunk is seldom detected in infants, even in those with cardiopathies with marked pulmonary hyperflow. This anatomical confirmation, in this case, was due to the direct flow from the left ventricle to the pulmonary artery tree, through the ventricular septal defect. Moreover, this defect, when doubly committed to the great vessels, pathogenically explains the concomitant existence of aortic coarctation, considering the increased Clinical dataThe patient remained asymptomatic up to three months before, when he started to present fatigue even at rest, which grew progressively worse.Physical examination: the patient was tachydyspneic, had normal skin color, ample pulses in both upper extremities and decreased in the lower extremities. Weight was 5,130g. The blood pressures in the right upper limb and right lower limb were 150/80 and 100/80 mmHg, respectively. HR: 130 bpm. The aorta was not palpated at the suprasternal notch. The patient presented slight precordial impulses at the left sternal border and the ictus cordis was diffuse and palpated at the 5th intercostal space on the hemiclavicular line. Heart sounds were hyperphonetic and there was a holosystolic...
Objetivo: Os procedimentos conservadores sobre a valva mitral têm vantagens sobre a troca da mesma. Avaliamos os resultados a médio prazo em pacientes com valvopatia mitral de causa reumática quanto às variáveis clínicas e ecocardiográficas. Casuística e Métodos: Cinqüenta e seis pacientes foram submetidos a procedimentos conservadores sobre a valva mitral de etiologia reumática. Quarenta e seis (82,1%) pacientes eram do sexo feminino e 10 (17,9%) do sexo masculino. A idade média dos pacientes foi de 34,70 anos (desvio padrão 13,88 anos). O tempo médio de seguimento foi de 23,84 meses (desvio padrão 9,23 anos). Vinte e cinco (44,6%) pacientes estavam em classe funcional III ou IV e 11 (19,6%) pacientes apresentavam fibrilação atrial antes da cirurgia. Os procedimentos realizados foram comissurotomia com papilaromiotomia em 11 (19,6%) pacientes, comissurotomia com anuloplastia utilizando órtese maleável de pericárdio bovino em 27 (48,2%) pacientes e anuloplastia com órtese maleável de pericárdio bovino em 18 (32,1%) pacientes. Resultados: Houve um óbito (1,7%) per-operatório (30 dias) no grupo estudado. Um paciente apresentou acidente vascular transitório no período pós-operatório, com total recuperação. Ocorreu melhora quanto ao volume diastólico final do ventrículo esquerdo de 52,0 ± 12,4 mm para 48,8 ± 9,9 (p = 0,001), tamanho do átrio esquerdo de 53,0 ± 8,0 mm para 47,8 ± 7,6 mm (p < 0,0001). Ocorreu melhora significativa quanto à classe funcional (p = 0,0001), ficando apenas 1 (1,8%) paciente em classe III. Ocorreram duas reoperações com 28,5 meses e 2,93 meses de seguimento; ficaram livres de reoperação 96,43% no tempo de seguimento. Conclusão: Concluímos que o reparo da valva mitral a médio prazo proporciona bons resultados, com melhora de parâmetros ecocardiográficos e clínicos.
Objective: Conservative procedures on the valve mitral have advantages over its replacement. We evaluated the mid term results in patients with rheumatic mitral disease regarding clinical and echocardiography variables. Material and Methods: Fifty six patients with rheumatic etiology were submitted to mitral valve repair. Forty six patients were female (82.1%) and ten male (17.9%). The average age was 34.70 years (standard deviation 13.88 years). Average time of follow-up was 23.84 months (standard deviation 9.23 years). Twenty five patients (44.6%) were in functional class III or IV and eleven patients with atrial fibrillation (19.6%) before surgery. The procedures were commissurotomy, commissurotomy with papillaromyotomy in 11 patient (19.6%), commissurotomy with malleable annuloplasty bovine pericardium prosthesis in 27 patients (48.2%) and malleable annuloplasty bovine pericardium prosthesis in 18 patients (32.1%). Results: There was one death (30 days) in this series (1.7%). One patient presented transitory vascular accident in the operative period with total recovery. Improvement ocurred with regard to the final diastolic volume of the left ventricule from 52.0 ± 12.4 mm to 48.8 ± 9.9 (p = 0.001), left atrium size from 53....
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