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PubMed1993

Aortic/mitral obstruction and coarctation of the aorta.

The prevalence, anatomy and physiology, clinical manifestations, laboratory findings, and management of patients with aortic/mitral obstruction and coarctation of the aorta are assessed. Subaortic stenosis reviewed includes discreet membranous and tunnel subaortic stenosis as well as hypertrophic obstructive cardiomyopathy. The discussion of valvular aortic stenosis includes both congenital and acquired forms of the disease. Supravalvular aortic stenosis is described along with coarctation of the aorta. Mitral valvular stenosis includes the congenital as well as acquired variety with focus on the congenital lesion. Current opinion regarding the medical, the cardiac catheterization, interventional, and the surgical approaches to therapy is presented.

Aortic Coarctation↗
PubMed1992

Effects of pacing tachycardia and balloon valvuloplasty on pulmonary artery impedance and hydraulic power in mitral stenosis.

BACKGROUND: Mitral stenosis is characterized by progressive pulmonary hypertension and eventual right ventricular failure. However, the correlation between right ventricular failure and the level of pulmonary hypertension is poor, suggesting that factors other than those recognized from nonpulsatile hemodynamic parameters may contribute to impaired right ventricular performance in this condition. METHODS AND RESULTS: We studied 16 patients with severe mitral stenosis (mean valve area, 1.0 +/- 0.2 cm2) at supine rest and during pacing tachycardia using high-fidelity catheter recordings of pulmonary artery (PA) pressure and flow velocity. Pulmonary impedance spectra, wave reflection properties, and hydraulic power data were derived from Fourier analysis of signal-averaged data. Pacing tachycardia (baseline heart rate, 81 +/- 11 beats per minute; pacing, 132 +/- 11 beats per minute) significantly raised pulmonary wedge and mean PA pressures. There was no change in pulmonary vascular resistance (209 +/- 144 to 232 +/- 164 dyne-sec/cm5) or PA characteristic impedance (62 +/- 25 to 55 +/- 28 dyne-sec/cm5). However, first harmonic impedance (Z1) significantly decreased (134 +/- 71 to 100 +/- 68 dyne-sec/cm5; p < 0.001). Accordingly, oscillatory and total dissipated hydraulic power per unit forward flow (WT/CO) fell during tachycardia (2.6 +/- 1.6 to 2.3 +/- 1.4 mW/ml.sec-1; p = 0.06) despite acute pulmonary hypertension. Reflected pressure waves returned earlier to the proximal PA, suggesting increased vessel stiffness. Immediately after percutaneous balloon mitral valvuloplasty (PBV) in eight of the patients, baseline and pacing data were again recorded. Compared with the pre-PBV baseline state, post-PBV resting data demonstrated no change in resistance or characteristic impedance, but there was a significant fall in Z1 (166 +/- 75 to 103 +/- 45 dyne-sec/cm5; p < 0.05) and in the magnitude of pulmonary wave reflections. WT/CO tended to decrease after PBV, and pacing after PBV produced a further decrease in WT/CO, again in association with lower Z1. CONCLUSIONS: These data demonstrate that 1) increased pulmonary characteristic impedance, although a feature of mitral stenosis, is not exacerbated by the acute effects of increased distending pressure; 2) pacing tachycardia in mitral stenosis causes little change in the pulmonary impedance spectrum except at low frequencies, where decreased impedance lowers power requirements per unit flow; and 3) relief of mitral stenosis produces immediate improvement in low-frequency impedance and in hydraulic power requirements. These findings suggest that although characteristic impedance may be a measure of the long-term effects of pulmonary hypertension on the pulmonary circulation, acute increases and decreases in PA pressure produce effects on right ventricular load that are best described in terms of the low-frequency properties of the PA system. Improvement in low-frequency impedance diminishes hydraulic power requirements and thus reflects improved ventricular-vascular coupling, irrespective of distending PA pressure. Efforts to treat or prevent right heart failure in the presence of pulmonary hypertension should take account of the potential benefit of changes in low-frequency impedance characteristics of the pulmonary vascular bed.

Adult↗
PubMed1992

Long-term follow-up results after reconstruction of the mitral valve by leaflet advancement.

In this study, we discuss the clinical results of mitral leaflet advancement performed on 29 patients over the past 10 years and attempt to determine the indication. Preoperative diagnosis of mitral valve lesion consisted of mitral regurgitation in 21 patients and mitral stenosis in 8 patients. Mitral valve repair was applied to the anterior mitral leaflet in 2, the posterior mitral leaflet in 25, and bilateral leaflets in 2 patients. Reoperation was performed on 13 patients, and 1 patient died of renal failure immediately after reoperation. No reoperation was needed for 96.6% of the patients at 1 year, 89.5% at 5 years, 75.0% at 8 years, 63.8% at 10 years, and 52.6% at 15 years postoperatively. At reoperation, the repaired mitral leaflet was found to be calcified in 3 patients more than 9 years after the initial operation. Of the 12 survivors without reoperation, mitral stenosis associated with regurgitation was obvious in 6 patients. Of the 21 patients with preoperative mitral regurgitation, 90.0% showed no deterioration at 5 years, 79.7% at 8 years, and 69.1% at 10 years. On the other hand, for the 8 patients with mitral stenosis, the rates were 87.5% at 1 year, 62.5% at 5 years, 50.0% at 8 years, and 25% at 10 years. Our results suggest that mitral leaflet advancement shows satisfactory results in patients with mitral regurgitation but is not successful for patients with mitral stenosis in the long term because the repaired valve tends to be stenotic in the late postoperative period.

Adolescent↗
PubMed2003

Progression from compensated hypertrophy to failure in the pressure-overloaded human heart: structural deterioration and compensatory mechanisms.

BACKGROUND: The progression of compensated hypertrophy to heart failure (HF) is still debated. We investigated patients with isolated valvular aortic stenosis and differing degrees of left ventricular (LV) systolic dysfunction to test the hypothesis that structural remodeling, as well as cell death, contributes to the transition to HF. METHODS AND RESULTS: Structural alterations were studied in LV myectomies from 3 groups of patients (group 1: ejection fraction [EF] >50%, n=12; group 2: EF 30% to 50%, n=12; group 3: EF <30%, n=10) undergoing aortic valve replacement. Control patients were patients with mitral valve stenosis but normal LV (n=6). Myocyte hypertrophy was accompanied by increased nuclear DNA and Sc-35 (splicing factor) content. ACE and TGF-beta1 were upregulated correlating with fibrosis, which increased 2.3-, 2.2-, and 3.2-fold over control in the 3 groups. Myocyte degeneration increased 10, 22, and 32 times over control. A significant correlation exists between EF and myocyte degeneration or fibrosis. Ubiquitin-related autophagic cell death was 0.5 per thousand in control and group 1, 1.05 in group 2, and 6.05 per thousand in group 3. Death by oncosis was 0 per thousand in control, 3 per thousand in group 1, and increased to 5 per thousand (groups 2 and 3). Apoptosis was not detectable in control and group 3, but it was present at 0.02 per thousand in group 1 and 0.01 per thousand in group 2. Cardiomyocyte mitosis was never observed. CONCLUSIONS: These structure-function correlations confirm the hypothesis that transition to HF occurs by fibrosis and myocyte degeneration partially compensated by hypertrophy involving DNA synthesis and transcription. Cell loss, mainly by autophagy and oncosis, contributes significantly to the progression of LV systolic dysfunction.

Aged↗
PubMed1993

Plasma noradrenaline as an indicator of functional state in hearts with mitral stenosis: the influence of acutely reduced left atrial pressure by balloon mitral commissurotomy.

To investigate the mechanism in which plasma noradrenaline concentration (pNA) is elevated in heart failure, the effect of balloon mitral valvuloplasty was used as a model of acute manipulation of the left atrial pressure reduction in ten patients with mitral stenosis. Gorlin mitral valve area and pNA were correlated with New York Heart Association functional class and found to have a significant exponential inverse relationship with each other ([pNA, pg/ml] = 198.9 x [mitral valve area, cm2]-0.696; P = 0.003). Elevated pNA could be partially explained by a reduced cardiac index (CI) ([pNA, pg/ml] = 403.4 x [CI, l/min/m2]-0.889; P = 0.027; r = 0.495), especially in severely failed hearts, but not by pulmonary capillary wedge pressure (PCWP). However, the percent changes (% delta) of variables early after balloon valvuloplasty exhibited a paradoxical contrast; % delta pNA showing a clear negative exponential correlation with % delta PCWP ([% delta pNA] = 436.0 x [% delta PCWP + 80]-0.679 - 80; P = 0.021), but not with % delta CI. These results suggest that pNA should be considered an indicator of cardiac functional class in mitral stenosis. PNA is modulated by both cardiac index and pulmonary capillary pressure, but in different ways.

Adult↗
PubMed2003

Perioperative management of pregnant patients with heart disease for caesarian section under anaesthesia.

One hundred pregnant patients, of age group 22 to 35 years, with different types of cardiac ailments (mitral stenosis, mitral regurgitation, mitral valve prolapse, aortic regurgitation, atrial septal defect, ventricular septal defect, coarctation of the aorta, Eisenmenger syndrome, hypertrophic obstructive cardiomyopathy and operated tetralogy of fallot), put up for elective caesarian section underanaesthesia, were managed in the department of anaesthesiology at IPGME&R/SSKM Hospital, Kolkata from January 1996 to December 2002. The aim of the study was to observe the maternal and foetal outcome in different heart diseases. Death occurred in 2 patients (67%) with Eisenmenger syndrome, in one patient (20%) with hypertrophic obstructive cardiomyopathy and in one patient (5%) with critical mitral stenosis (mitral orifice area = 0.6 cm2) with pulmonary arterial hypertension (PAH). Neonatal mortality was observed in 4 patients [Eisenmenger syndrome--3 (100%); coarctation of the aorta--1 (33%)]. Another 8 patients developed severe heart failure (HF) [severe mitral stenosis (mitral orifice area = 1-1.2 cm2)--2 (10%); hypertrophic obstructive cardiomyopathy--4 (80%); coarctation of the aorta--2 (66%)]. Foetal dysmaturity was observed in 20 neonates (54%) belonging to mothers of New York Heart Association (NYHA) classes III and IV. Congenital heart disease (ventricular septal defect) was detected in 3 offsprings (20%) of mothers with ventricular septal defect. The study concludes that most pregnant cardiac patients can have a satisfactory outcome with careful perioperative management.

Adult↗
PubMed2001

[Optimal medical care for children with Down syndrome and their parents].

Children with Down's syndrome have an increased risk of congenital anomalies and additional medical problems. These can be treated but are often seen as part of the syndrome and not as the cause of developmental retardation. Based on the current level of knowledge, specific medical tests and evaluations are recommended. The reasons for these guidelines, composed by the Down's syndrome Work Group of the Paediatric Association of the Netherlands, are illustrated by the case reports of five patients: in a 4-year-old boy, retardation improved after celiac disease had been diagnosed. In a 5-year-old boy, slow speech development was reversed after hearing loss was diagnosed. In a 2-year-old girl, development improved after a mitral valve stenosis had been corrected. In a 6-month-old boy, drinking problems were reversed when torticollis was diagnosed and a self-deflating drinking bottle was introduced. An adult man suffering from epilepsy and from self-mutilative rubbing and hitting of his eyes, regained his sight after cataract, myopia and atrophy bulbi were diagnosed. A well-balanced disclosure of the diagnosis, repeated medical screening, early motor training and social interventions combined with active parental participation, warrant optimal development. In cooperation with the Down's syndrome Parent Association, multidisciplinary medical teams have been established to offer and promote this care.

Adult↗
PubMed1990

Echocardiography. Basics for the primary care physician.

Echocardiography is commonly performed to assess aortic stenosis, mitral valve prolapse, pericardial effusion and cardiac tamponade, myocardial infarction, endocarditis, and valvular regurgitation. We have found that the more defined and appropriate a request for an echocardiogram is, the better the technician and interpreter can relate observations to the requesting physician. Thus, thorough understanding of the indications and limitations of echocardiography by the primary care physician will result in improved patient care.

Aortic Valve Stenosis↗
PubMed1975

Pulmonary edema following pulmonary valvulotomy.

A previously unreported combination of critical pulmonary valve stenosis and mitral stenosis is described. The initial clinical presentation was one of right ventricular failure that obscured the evidence of pulmonary venous hypertension. Following pulmonary valvulotomy, pulmonary edema ensued because of the increased pulmonary blood flow. The importance of urgent cardiac catheterization postoperatively following an operation that increases pulmonary blood flow is discussed.

Child↗
PubMed1996

[A case report of a left atrial ball thrombus at 16 years after mitral valve replacement].

A free floating ball thrombus in left atrium is very rare, only 2 cases that develop ball thrombus after mitral valve replacement (MVR) have been reported. This case was a 55-year-old female who underwent MVR and tricuspid annuloplasty (TAP) in 1978. After 16 years, she developed replaced mitral valve stenosis due to pannus formation and tricuspid regurgitation (TR), with a free floating ball thrombus in the giant left atrium. A ball thrombus was taken out, and Re-MVR and tricuspid valve replacement (TVR) was carried out. Post operative course was uneventful. Because a free floating ball thrombus easily causes "Hole-in-one" sudden death, early surgical treatment must be considered.

Female↗
PubMed1976

[Calculation of the mean pressure of the pulmonary artery and of the valve surface by ECG in mitral stenosis].

From a group of 50 patients affected by pure mitral stenosis with sinus rhythm, we have tried to obtain multiple correlation equations which enable in to calculate, in each particular case, the mean pressure of the pulmonary artery and mitral valvular area from purely electrocardiographic facts. In an additional group of 16 patients we have been able to verify the mentioned equations, placing all the calculated points within the presumed limits of tolerance. So it appears that the electrocardiographic calculation of these hemodynamic parameters is possible, with sufficient approximation for clinical requirements.

Blood Pressure Determination↗
PubMed1985

Modified technique of transseptal left heart catheterization.

Transseptal left heart catheterization was performed in 106 instances in 101 patients using right anterior oblique fluoroscopy to define septal boundaries during interatrial septal puncture, and using a preshaped guide wire to catheterize the left ventricle. By using these two modifications of the classic transseptal technique, the left atrium was entered in 105 instances (99%) and the left ventricle was catheterized in all 87 attempts (100%), including attempts in eight patients with mitral stenosis (valve area 1.29 +/- 0.39 cm2 [mean +/- standard deviation] ). No deaths occurred as a direct result of transseptal catheterization; nonfatal complications occurred in 2.8% of patients (hemopericardium in one patient, ventricular fibrillation in one patient and transient vagal reaction in one patient). The use of the right anterior oblique projection to adequately visualize both the interatrial septum and the intended point of puncture, the use of a pigtail catheter positioned in the ascending aorta to define the relation of the puncture site to the aorta in this projection and the utilization of a flexible preshaped guide wire to catheterize the left ventricle are the major factors contributing toward this improved success rate and low incidence of complications.

Adult↗
PubMed1980

Extracoronary collateral myocardial blood flow during cardioplegic arrest.

Extracoronary blood flow to the myocardium was studied in 54 patients during cold cardioplegic arrest. Coronary venous return was measured with the aorta and the pulmonary artery cross-clamped, both venae cavae occlusively snared, and the heart completely drained. Cold St. Thomas' cardioplegic solution was infused into either the aortic root or the coronary ostia. Myocardial septal temperature was continuously monitored. The amount of blood in the right atrial effluent was determined by means of the hematocrit and was considered to be the extracoronary collateral myocardial blood flow (QE), originating from the systemic circulation. QE ranged from 0 to 1470 ml-100 min-1 (x = 241.1 ml-100min-1). The myocardial spontaneous rewarming rate was not significantly correlated to QE. QE was lowest in pure mitral valve stenosis (x = 39.9 ml-100 min-1) and higher in aortic valve disease (x = 165.5 ml-100 min-1). Very high QE values (greater than 800 ml-100 min-1) were only observed in patients with severe three vessel coronary artery disease. Patients with angina at rest appear to have lower QE values than patients with equally severe coronary artery disease suffering from angina under excise only. It is concluded that extracoronary collateral blood flow may unpredictably influence the efficacy of clinical cardioplegia and may to some extent compensate for severe coronary artery disease.

Aortic Valve↗