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Balloon valvuloplasty in congenital and acquired heart disease: morphologic considerations.

Balloon valvuloplasty has already gained its place as an alternative option in the treatment of valvar stenoses. In congenital valvar stenosis its application will be limited because of the complexity of the underlying valve deformities. Nevertheless, isolated pulmonary and aortic valve stenosis are amenable to this form of treatment. The major limitations are undeveloped arterial valve rings and valve dysplasia. In acquired valvar stenosis the main limitation of balloon valvuloplasty seems to be massive calcification of the affected valves. Basically, in both the arterial and atrioventricular position, balloon valvuloplasty splits fused commissures and fractures calcific deposits. Excessive radial pressures may cause tears of the leaflets. The potential of thromboembolic complications, therefore, warrants caution. The nature of the damage inflicted on pathologic valves almost by necessity dictates that restenosis will occur and - most likely - with a high incidence.

Aortic Valve Stenosis↗

Pulmonary valvular stenosis associated with Takayasu's Disease. Favorable response to corticosteroids. A case report.

The authors describe the first reported case of type IV Takayasu's arteritis with pulmonary valve stenosis. After thirty months under corticosteroid therapy the disappearance of the pulmonary valve stenosis signs was observed in the patient. In the same patient coarctation of the aorta, aortic insufficiency, stenosis of both pulmonary arteries, and left coronary artery stenosis were observed. This case illustrates the extensive cardiovascular involvement that can occur in Takayasu's arteritis and suggests that pulmonary valvular stenosis could be secondary to the same inflammatory process.

Adult↗

[Left ventricular hypertrophy simulating an intracavitary tumor. Echocardiographic diagnosis].

We report a case of a 14 month old infant with severe stenosis of the pulmonary valve in whom the angiographic image suggested the presence of a right ventricular (RV) tumor mass. The patient was admitted because of clinical manifestations of heart failure. Right heart catheterization demonstrated a transvalvular pulmonary gradient and filling defects in the infundibulum compatible with an intracavitary mass. Two dimensional and Doppler echocardiography confirmed pulmonary stenosis and showed that the mass corresponded to hypertrophy of the anterior RV wall. The combination of two dimensional and Doppler echocardiography are the indicated techniques in differential diagnosis of RV tumor masses and RV hypertrophy secondary to severe pulmonary valve stenosis.

Cardiomegaly↗

Percutaneous balloon pulmonary valvuloplasty.

Percutaneous pulmonary valvuloplasty was performed in 27 patients with congenital pulmonary valve stenosis. A fall in the transvalve gradient of at least 15 mm Hg occurred in 22 patients. In five there was little change in the severity of the stenosis; in three of these the pulmonary valve was dysplastic. None of the successfully treated patients had a dysplastic valve. The two other failures, early in the series, were probably due to inadequate balloon size. In one patient the procedure was performed twice, with a successful result from the second dilatation with a larger balloon. Follow up studies in a further six patients showed no evidence of restenosis in those who had been successfully treated and no late improvement in the remainder. There were no important complications. Percutaneous pulmonary valvuloplasty should be the initial treatment for congenital pulmonary valve stenosis, although when the valve is dysplastic the result is less likely to be satisfactory.

Adolescent↗

Triple valve replacement in a patient with severe carcinoid heart disease.

We report on the case of a 34-year-old male patient suffering from end-stage carcinoid heart disease with severe tricuspid, pulmonary and mitral valve regurgitation. In addition, a persisting foramen ovale was present. The primary carcinoid tumor was never discovered. However, urine 5-hydroxy-indole-acetic-acid (5-HIAA) were consistently elevated after the first diagnosis of carcinoid disease and after eight years of medication with Octreotide and Interferon alpha-2b our patient developed significant cardiac insufficiency mainly due to severe valvular dysfunction. Ultimately, mechanical tricuspid, mitral and pulmonary valve replacement was performed. Twelve hours following the operation the patient had to be returned to the operating room for persisting intrathoracic hemorrhage. He recovered uneventfully and was discharged from hospital on day 37. Twelve months following triple valve replacement the cardiac status recovered from preoperative NYHA-IV to NYHA-I.

Adult↗

Left ventricular approach to multiple ventricular septal defects.

Multiple muscular ventricular septal defects were closed through an apical left ventriculotomy in 11 patients. The patients were divided into two groups: Group 1, 8 patients who had transposition of the great arteries, and group 2, 3 patients without transposition. There were 4 deaths in Group 1 and non in Group 2. Two of the deaths were caused by a hypoplastic right ventricle, 1 by airway obstruction, and 1 by heart failure and pulmonary edema in a patient who had additional unrecognized muscular defects. An apical left ventriculotomy provides excellent exposure of the septum. The field is not obscured by trabecular bands or papillary muscles. Although 1 patient died because of residual VSDs, this approach, compared with previously described methods, minimizes the risk of unrecognized defects.

Animals↗

Pulmonary valvotomy under normothermic caval inflow occlusion.

Pulmonary valve stenosis may require urgent surgical relief in infancy or elective valvotomy in childhood. A retrospective study has been made of 94 children who underwent pulmonary valvotomy for pulmonary valve stenosis between 1972 and 1983 using the technique of normothermic caval inflow occlusion. There were no early deaths nor late deaths. The group included 13 neonates less than 1 week old and 14 other infants under 1 year of age. The mean follow-up is 45 months. No child has had a second valvotomy for recurrent valvar stenosis. Two children have required re-operation for placement of a transannular right ventricular outflow patch for hypoplastic pulmonary annulus. Neonates who present early with critical pulmonary valve stenosis may remain moderately to severely cyanosed for several days after a satisfactory valvotomy but this is almost invariably followed by a progressive increase in oxygen saturation to an acceptable level. One neonate, who had a Blalock-Taussig shunt at 2 weeks of age for persistent postvalvotomy cyanosis, had the shunt ligated at 2 years. Pulmonary valvotomy under normothermic caval inflow occlusion is a safe, cost-effective technique which provides excellent early and late haemodynamic results. This operation sets a standard against which the newly introduced technique of percutaneous balloon pulmonary valvotomy should be assessed.

Adolescent↗

[Cardiovascular dysplasia in Noonan's syndrome. Apropos of 64 cases].

The multiple malformation syndrome with characteristic facies described by Jacqueline Noonan in 1963 is one of the most commonly encountered syndromes in paediatric cardiology. The series presented here comprises 64 cases, almost a quarter of which were familial (6 families). The morphotype (Turner phenotype with hypertelorism) can easily be recognized, but the variations, progressivity and resemblance to some similar syndromes imply a detailed analysis of a wide range of discriminant features. Short stature and webbed neck are less frequent than in Turner's syndrome, while mental debility and, mostly, cardiovascular lesions are much more frequent (the latter are present in 50-60% of the cases). The most typical cardiovascular lesion is pulmonary valve stenosis with dysplastic leaflets, or "atypical pulmonary stenosis syndrome", characterized by a curious electrical axis and above all, by left ventricular myocardiopathy with very peculiar deformations at ventriculography and 2D-echocardiography. In our series, myocardiopathy was even more frequent than dysplastic pulmonary valve stenosis (71.8% versus 64%). The dissemination of dysplastic lesions must be emphasized. They involve the aorta much more often than is usually reported (one-third of the cases); lesions of the lymphatic system are less frequent but may be severe. Occasionally, entirely different heart diseases, such as Fallot's tetralogy, are encountered. Owing to the relationship between its lesions and those of other multiple malformation syndromes, notably those of the phakomatosis group, and to the possibility of borderline cases with these syndromes, or even with other dystrophies, such as Williams-Beuren dystrophia, the cardiovascular dysplasia of Noonan's syndrome must be classified within the vast group of histodysplasias which are embryonic diseases of the "layers" or "neuro-ecto-mesodermoses" of unknown genetic mechanism.

Adolescent↗

Congenital absence of the pulmonary valve associated with pulmonary stenosis, large ductus arteriosus and intact ventricular septum. Case report.

A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.

Ductus Arteriosus, Patent↗

Assessment of valvular heart disease by Doppler echocardiography.

Doppler echocardiography provides direct hemodynamic data that are often complementary to those demonstrated by M-mode and two-dimensional echocardiographic imaging. This relatively new noninvasive technique has a number of important uses in patients with valvular heart disease. In both adults and children, Doppler measures of peak flow velocity through a stenotic valve allow accurate prediction of the pressure gradient across the valve, and the technique has particular promise for screening patients with suspected aortic or pulmonic stenosis. In patients with mitral stenosis but parasternal short-axis images of limited quality, Doppler velocity measures can provide novel data about the pressure gradient and mitral orifice area. Doppler techniques can also provide direct evidence for or against the presence of valvular regurgitation, and several approaches allow clinically useful estimation of the extent of aortic, mitral, or tricuspid regurgitation. In patients with known disease of one cardiac valve, Doppler is accurate for evaluating the integrity of a second valve. Finally, Doppler techniques have great promise for defining the nature, and perhaps the severity, of suspected prosthetic valve malfunction. Hence, we believe that Doppler echocardiography should become a routine part of the noninvasive evaluation of patients with known or suspected valvular heart disease.

Aortic Valve Insufficiency↗

Balloon valvuloplasty 1988: a review.

Catheter balloon valvuloplasty is a procedure which has been applied in stenotic lesions of all four cardiac valves in the last several years. This review discusses available data regarding the mechanism of effect, risks, and clinical utility of balloon valvuloplasty as it is applied to stenotic lesions of the pulmonic, mitral and aortic valves.

Aortic Valve Stenosis↗