Primary pulmonic infundibular stenosis in 12 cats: natural history and the effects of balloon valvuloplasty.

OBJECTIVES: To evaluate the natural history of primary pulmonic infundibular stenosis in cats and the effects of balloon valvuloplasty. BACKGROUND: Primary pulmonic infundibular stenosis is an uncommon congenital defect in cats. The natural history of the disease has not been described. Information regarding balloon valvuloplasty in the cat is limited. ANIMALS: Records between January 1, 1999 and December 31, 2005 were reviewed and cats with a confirmed echocardiographic diagnosis of primary pulmonic infundibular stenosis, a complete medical history, and no evidence of significant systemic disease were identified. METHODS: Echocardiographic, electrocardiographic, and radiographic findings are described. The natural history of those with severe disease was compared to those with mild to moderate disease. Balloon valvuloplasty was performed in six of the cats. The technique used is described. RESULTS: A stenotic gradient >or=70 mmHg and a right ventricular outflow tract (measured at the level of the stenosis) to pulmonary valve annulus ratio of

Masa hiliar izquierda en paciente con estenosis valvular pulmonar / Enlargement left hilar in a man with pulmonary valvular stenosis

Se presenta el caso de un paciente varón de 38 años deedad con antecedente de estenosis pulmonar como hallazgo,asintomática y sin tratamiento, que se confirmó enla exploración física. Se muestran Rx Tórax, TAC torácica y ecodoppler cardiológico.Se analizan las características radiológicas de la estenosis pulmonar y la habitual asociación de ésta con crecimiento de las ramas principales de la arteria pulmonar.Se establece diagnóstico diferencial entre las diversas entidades responsables de agrandamiento hiliar izquierdo

A case of 38 years old man with pulmonary stenosis finding, without traitment and no symtomatology we present. Chest Radiographs, Thoracic TAC and echocardiography has shown.The radiologic features to the pulmonary stenosis and theasociation with enlargement of the main branches pulmonary artery were analysed.We reviewed the different entities responsible of enlargement left hilar

Pseudo-Bartter syndrome in a neonate on prostaglandin infusion.

UNLABELLED: We describe a case of iatrogenic pseudo-Bartter syndrome caused by administration of prostaglandin E1 (PGE1 alprostadil). Although the use of i.v. PGE1 is a well-established pharmacological therapy in neonates with a ductus-dependent congenital cardiopathy to ensure ductus-dependent flow, we could only find one other report on pseudo-Bartter syndrome related to PGE1 infusion. CONCLUSION: Primary Bartter syndrome is associated with endogenous increased levels of prostaglandins. Therefore, we postulate that the dose of prostaglandin E1 administered, immaturity and the genetic background are all relevant factors involved in the phenotypic presentation of iatrogenic pseudo-Bartter syndrome in this preterm infant.

[Intermediate and long-term outcome after percutaneous balloon dilatation of valvular pulmonary stenoses in childhood]. / Mittel- bis langfristige Ergebnisse nach perkutaner Ballondilatation valvulärer Pulmonalstenosen im Kindesalter.

Pulmonary balloon valvuloplasty was performed in 52 patients aged 7 days to 19 years (mean 5.5 years). Mean balloon/anulus ratio was 1.24. Mean right ventricular outflow tract (RVOT) systolic pressure gradient was 79.9 +/- 37.3 (x +/- SD) mm Hg before valvuloplasty and 37.2 +/- 29.6 mm Hg (p < 0.001) immediately after the procedure. 33 patients had residual RVOT-gradient < or = 36 (22 +/- 7) mm Hg, 19 patients had gradients > 36 (67.1 +/- 35.6) mm Hg. During intermediate follow-up (< 2 years) RVOT gradient assessed by catheterization or Doppler echocardiography improved without any additional intervention in 10/19 patients with RVOT gradient > 36 mm Hg early after valvuloplasty. 6/19 patients required additional reduction of RVOT gradient by repeated valvuloplasty (51 +/- 19 to 29 +/- 5 mm Hg; p < 0.01). 3/19 patients needed surgical resection of extremely thickened dysplastic valves. 49 patients had a mid- to long-term follow-up by echocardiography (mean 4.3, max. 9 years); the RVOT gradient decreased from 25.7 +/- 12.8 mm Hg during intermediate follow-up to 18.0 +/- 7.0 mm Hg during long-term follow-up. If pulmonary regurgitation was present after dilatation (n = 38), it was hemodynamically not significant and did not change during follow-up. Percutaneous balloon valvuloplasty was a safe and effective treatment for pulmonary valve stenosis in infancy and childhood. Long-term results confirm the value of this method.

Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults.

Congenital pulmonary valve disease is often not discovered until adolescence or adulthood. Transthoracic two-dimensional echocardiography can provide detailed information regarding right ventricular outflow anatomy, although images are often less satisfactory than those obtained in infants and children. The more recent addition of biplanar transesophageal echocardiography has enhanced our ability to image the right ventricular outflow tract, pulmonary valve, and pulmonary artery noninvasively. Pulsed and continuous-wave Doppler estimates of subvalvular and transvalvular gradients have proved to be accurate. Doppler color flow mapping has proved useful in determining the location and direction of stenotic and regurgitant flow. With no accepted standard for comparison, quantification of regurgitation remains problematic. In many cases, echocardiography has replaced catheterization and angiography in the evaluation and long-term follow-up of congenital pulmonary valve disease before and after intervention.

Univentricular atrioventricular connection to a dominant left ventricle with a concordant ventriculo-arterial connection.

Double inlet left ventricle with concordant ventriculo-arterial connection, first described by Holmes, is an unusual cardiac malformation which includes a right-sided rudimentary right ventricle and, frequently, subpulmonary stenosis. We have now encountered six patients, aged 1 month to 13 years, with this basic combination. In 5 cases, cross-sectional echocardiography, using parasternal, apical and subcostal views was diagnostic. Subpulmonary stenosis was seen in two of them. The sixth case shown to have atresia of the left atrioventricular valve, a concordant ventriculo-arterial connection, a left-sided rudimentary right ventricle, mild subpulmonary stenosis and a right-sided aorta. The combination of the left atrioventricular valve, left-sided rudimentary right ventricle and a concordant ventriculo-arterial connection has not, to our knowledge, been previously reported. Cross-sectional echocardiography always provided the correct morphologic diagnosis.

An autopsy case of sudden unexpected death due to atherosclerotic coronary heart disease associated with simple right ventricular outflow tract stenosis.

A case of a 35-year-old male who died suddenly after a blow on the chest by his opponent during a quarrel. From both the clinical and autoptical examinations there was no evidence of trauma found anywhere, even in the chest. The heart was the only one of all the internal organs whereon significant pathologic changes were observed during autopsy. It's lesions were as follows: (I) The right ventricle was greatly enlarged simply due to the simple right ventricular outflow tract stenosis (SRVOTS); the heart weight was 587 g. (II) All the main trunks of coronary arteries were involved by atherosclerosis. In addition, the appearance of the lumen at the proximal end of the left anterior descending artery narrowed by an atheromatous plaque (grade II) was completely occluded by a newly formed thrombus. The authors believed that both the coronary atherosclerotic changes and the enlarged heart due to the SRVOTS played a role in the pathogenesis of death (Kelberlus, H.E. and Wellens, H.J.J., Sudden Death, Developments in Cardiovascular Medicine, Vol. 4, Martinys Nijhoff Publishers, London, 1980, pp. 34-48; Jinming, Qin, A study of cardiac sudden death (51 cases) on the clinical pathologic view, Tianjin Med., 8 (1980) 458-461). The quarrel and blow were the disposing factor.

Non-invasive diagnosis of subpulmonary outflow tract obstruction.

We have studied the echocardiographic and phonocardiographic findings in 18 patients with obstruction to ventricular outflow at subpulmonary valve level. The aetiology was congenital in 13 patients, a result of hypertrophic cardiomyopathy in three, and infiltration of the right ventricular outflow tract by glycogen or lymphoma in the remaining two. Abnormal systolic motion of the pulmonary valve, fluttering, and early or midsystolic closure were seen in 16 of 17 patients in whom the cusps were visualised. Normal pulmonary valve motion was found in one patient with coexisting pulmonary valve stenosis. In congenital infundibular stenosis the delay of the pulmonary component of the second heart sound (P2) was related to the severity of the obstruction. A pulmonary ejection sound, defined as a high-frequency sound occurring at the moment of full pulmonary valve opening, was absent except in the patient with coexisting pulmonary valve stenosis. In hypertrophic cardiomyopathy with obstruction to the right ventricular outflow, the ejection systolic murmur was softer with inspiration, a finding that contrasts with the respiratory variation seen with fixed obstruction. Recognition of these abnormalities should allow an accurate non-invasive diagnosis to be made and permit assessment of severity when P2 can be recorded.

Surgical correction of unusual double-outlet right ventricle.

This paper presents the case history of an 8-year-old girl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a Bjork-Shiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examination revealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made.