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At least 793 records · Page 44Linked to original sources
PubMed1990

Management of neonatal critical pulmonic stenosis in the balloon valvotomy era.

We evaluated our recent experience with management of neonatal critical pulmonic stenosis and intact ventricular septum between 1982 and 1988. Thirty-nine patients (aged less than 3 months) were treated initially by operation (group A, n = 19) or with balloon pulmonary valvotomy (group B, n = 20). Patients in group A were younger (5 +/- 1.3 versus 18 +/- 4 days in group B) (mean +/- standard error of the mean) and had a greater degree of hypoxia (oxygen tension, 55 +/- 4 versus 80 +/- 6 mm Hg) (p less than 0.05 for all variables). Ten patients in group A and 8 patients in group B had right ventricular hypoplasia, based on an angiographically determined index. Balloon pulmonary valvotomy was attempted in 20 patients at the time of the initial catheterization but was unsuccessful in 9 owing to inability to catheterize the hypoplastic right ventricular outflow tract (n = 8) and to recurrent infundibular stenosis (n = 1). Patients with failed balloon valvotomy were subsequently operated on within 24 hours. The early operative mortality (less than 30 days) was 25% (7 of 28); one death (9%) occurred after successful balloon valvotomy owing to associated critical aortic stenosis. The early postoperative gradient was 20 +/- 2 mm Hg; the post-balloon valvotomy gradient was 18 +/- 3 mm Hg. We conclude that balloon pulmonary valvotomy yields good results in patients with critical pulmonary stenosis with essentially normal-sized right ventricle, whereas surgical pulmonary valvotomy is required for patients with right ventricular hypoplasia.

Blood Pressure↗
PubMed1991

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.

Adult↗
PubMed1980

Cardiovascular status after repair by Fontan procedure.

Patients surviving a Fontan operation experience dramatic symptomatic improvement, but concern remains about the long-term results of this operation. The clinical course and postoperative hemodynamic findings in 5 long-term survivors of the Fontan procedure from our institution are presented. Attention is drawn to 3 patients who required reoperation: 1 immediately for residual mild pulmonary stenosis, 1 for late onset complete heart block, and 1 for conduit valve stenosis. Review of our patients and those described in the literature reveals that all have ascites and pleural effusions as a results of high venous pressures but that this is usually a temporary problem. Late onset of obstruction to right atrial emptying has been reported in several patients in addition to ours. This raises serious concerns about the long-term fate of cloth conduits and porcine valves in this application. Although normal sinus rhythm has been thought to be essential for adequate pulmonary perfusion in these patients, it is interesting that several patients have tolerated atrial tachyarrhythmias, junctional rhythms, and even complete heart block without serious ill effects. We conclude that the Fontan procedure is extremely effective in relieving symptoms at an operative risk that is now acceptable, but these patients require very careful long-term follow-up because they are subject to a number of long-term complications.

Adolescent↗
PubMed1978

Reconstructive valve surgery.

Despite continued refinement of heart valve prostheses, valve replacement carries risks of thromboembolic, mechanical, and infectious complications, and long-term success is further limited by the eventual wear of prosthetic parts. In many patients with congenital or acquired valve diseases, valve function may be improved, if not restored, by reconstructive techniques, prosthetic replacement being thereby avoided or delayed. This review examines the current status of reconstructive procedures for management of diseased valves, with emphasis on long-term results and post-operative hemodynamic studies. In many instances the choice between reconstruction and replacement of a diseased valve remains controversial. The documented success of selectively applied reconstructive techniques, however, weighs against expedient decisions for prosthetic replacement and supports a continuing search for new techniques.

Aortic Valve↗
PubMed1976

Corrected transposition of the great vessels: a vectorcardiographic study.

The vectorcardiograms (VCGs) of two groups of patients with corrected transposition of the great vessels (CTGV) were studied; the first, group A, included 17 patients with CTGV in "situs solitus,", characterized by leftward orientation of the cardiac apex; the second group, group B, with three patients, presented CTGV in "situs solitus" and apex to the right. All cases had one or more associated defects: ventricular septal defect, atrial septal defect, pulmonic stenosis or tricuspid insufficiency.

Action Potentials↗
PubMed1990

Geleophysic dysplasia: a storage disorder affecting the skin, bone, liver, heart, and trachea.

Geleophysic dysplasia is characterized by typical facies ("happy natured"), small hands and feet, short stature, hepatomegaly, and progressive cardiac disease. We describe five patients (two of whom are siblings) with this disorder and document its variable expressivity. The facies were strikingly similar with small nose, anteverted nostrils, broad nasal bridge, and long thin upper lip with flat and long philtrum. Behavior, development, and intelligence were normal. Growth delay was noticed during infancy, and the two patients who completed normal puberty had marked short stature (140 and 150 cm), with relatively lean body habitus. The hands and feet were small, with short, plump tubular bones and broad proximal phalanges, associated with marked limitation in motion of fingers and wrists. The liver was enlarged after the age of 3 years. Two patients had mild mitral and tricuspid valve stenosis and one had severe aortic stenosis. The most severely affected child died at 3 1/2 years of age of airway obstruction as a result of progressive tracheal narrowing. Lysosomal storage vacuoles were found in skin epithelial cells from three patients whose skin was examined, and in the tracheal mucosa, liver, cartilage and macrophages of the child who died. The basic defect of this autosomal recessive lysosomal storage disease remains to be determined.

Adolescent↗
PubMed2003

[Hand-held echocardiographic devices: what do they add to the initial cardiovascular evaluation?].

INTRODUCTION AND OBJECTIVES: Hand-held echocardiographic devices have recently become available. Our objective was to determine, on the basis of clinical data and basic diagnostic techniques, whether hand-held devices offer additional information useful not provided by the initial cardiovascular diagnosis. PATIENTS AND METHOD: We prospectively studied the presence and severity (absent, mild, significative) of 7 frequent heart diseases (aortic or mitral stenosis-regurgitation, tricuspid regurgitation, and left ventricular systolic dysfunction-hypertrophy) in 36 consecutive patients (50% men; mean age 68 12 years) with 3 different methods: clinical examination and basic complementary exams, hand-held echocardiography with 2D and color Doppler imaging (OptiGo, Philips Medical Systems, The Netherlands) and a standard, last-generation transthoracic echocardiogram (Sonos 550, Philips Medical Systems, The Netherlands). We compared the results obtained with the first two methods, and combined the results of both to compare these findings against the results obtained with standard electrocardiography. Percentage agreement and Somer's D, a measure of association between ordinal variables, were calculated. RESULTS: The hand-held device obtained better results than clinical examination (agreement 87 vs. 65%; D = 0.79 0.04 vs. 0.19 0.53) and identified severe lesions that were classified incorrectly by clinical examination in 39% (14/36) patients. However, in 8 patients (10 evaluations) it misclassified severe lesions. CONCLUSIONS: In experienced hands, a hand-held echocardiographic device offers additional information not obtained from an initial cardiovascular diagnosis for common cardiovascular disorders, but it is no substitute for complete echocardiographic examination.

Aged↗
PubMed2001

Double inlet right ventricle versus other types of double or common inlet ventricle: its clinical characteristics with reference to the Fontan procedure.

OBJECTIVE: To determine the clinical features in patients with double inlet right ventricle (DIRV) and separate atrioventricular (AV) valves in terms of the Fontan procedure. METHODS: Surgical procedures were carried out in 31 patients with this rare malformation. Their clinical characteristics were compared with similar features in 45 patients with double inlet left ventricle (DILV), 93 with common inlet right ventricle (CIRV), and 20 with common inlet left ventricle (CILV). RESULTS: Pulmonary atresia with the right and the left pulmonary arteries being confluent was seen in 58% of patients with DIRV, and pulmonary atresia with non-confluent pulmonary arteries in 15% (P=0.0001). The incidence of these findings was significantly higher when compared with DILV (P=0.0001). Reflecting these morphologic features, constructions of systemic-to-pulmonary shunts and extensive plasty to the pulmonary arteries were more frequently needed in patients with DIRV than in the others (P=0.04, 0.0001). The AV valves have become moderately or severely regurgitant in three with DIRV (10%). The Fontan procedure has thus been employed in 21 with DIRV (64%), this incidence being significantly higher than that seen in the CIRV (37%) group (P=0.01). Survival rate of 95% at 10 years in this group was as excellent as that in the DILV group, and obviously better than that in either CIRV or CILV (P=0.002). CONCLUSIONS: Although multiple palliative procedures might be frequently needed to maintain pulmonary perfusion, the Fontan circulation can be justifiably established in patients with DIRV, and regurgitation across the AV valves was not very common.

Child↗