Hereditary cardiovascular dysplasia

Pare, Jean-Pierre; Fraser, Robert G.; Pirozynski, W. J.; Shanks, John A.; Stubington, David

doi:10.1016/0002-9343(61)90222-4

Cited by 178 publications

(20 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The familial incidence in this and similar studies of hypertrophic obstructive cardiomyopathy, especially those of Pare et al (1961) and other reports of cardiomyopathies of familial origin, suggest that a genetic abnormality of cardiac muscle may be the basic cause. Possibly an inherited developmental error in the function of the deep bulbospiral muscle or in the growth of cardiac muscle and in catecholamine function may be the original abnormality that could lead to the irregular ventricular hypertrophy (Fig.…”

Section: Unclassified Groupsupporting

confidence: 79%

Cardiac Function in Primary Myocardial Disorders--Part II

Goodwin¹

1964

BMJ

View full text Add to dashboard Cite

The data presented indicate that myocardial function in primary cardiomyopathy falls into three main groups: congestive, constrictive, and hypertrophic obstructive. However, clear-cut differences between the congestive and constrictive groups do not always exist, though the hypertrophic obstructive group appears to be different. In the congestive and constrictive groups the absence of any obvious cause for the heart disease and the finding of round-cell infiltration and fibrosis at necropsy in some would harmonize with an infective origin for many, though the histological and virological studies gave no support for this. Nevertheless, myocarditis or infective cardiomyopathy has long been accepted as an entity. Possibly infection damages the delicate contractile elements of the myocardium and interferes with the biochemical and biophysical phenomena essential for efficient function. In some way also the toxic agent might prevent the hypertrophy that occurs readily with most other forms of heart disease.By contrast, the third group, hypertrophic obstructive cardiomyopathy, shows very marked differences from the other two, which strongly suggest another pathogenesis. A positive family history, massive ventricular hypertrophy, reduced ventricular compliance, disturbed contraction and systolic outflow gradients are associated with a high cardiac output and increased cardiac work.My colleagues and I do not entirely agree with Bjork et al.(1961), who maintain that in some cases abnormal attachment of the aortic cusps of the mitral valve may be a cause of obstruction to left ventricular outflow and of left ventricular hypertrophy.Our studies, and those of others, indicate that the abnormalities in hypertrophic obstructive cardiomyopathy result in impaired ventricular filling and abnormal ejection, in addition to mitral incompetence in many patients. In contrast with the other groups congestive cardiac failure is unusual and occurred in only one patient in our series, though dyspnoea may be common and is attributable to an increased left atrial pressure secondary to increased left ventricular end-diastolic pressure resulting from rigidity of the left ventricle. Presumably for similar reasons tricuspid incompetence is unusual, but augmented " a " waves are common in the jugular venous pulse, and in a number of patients are accompanied by outflow systolic gradients. The inflow obstruction is presumably due to massive hypertrophy in addition to abnormal ventricular compliance. In some patients evidence of outflow obstruction is lacking, the characteristic ejection murmurs being absent, and obstruction is confined to ventricular inflow. Thus the diagnosis may depend on the characteristic angiographic appearances already described. It is these patients, perhaps, who are closest to the intermediate or unclassified group which has already been presented and which may represent a hypertrophic preobstructive stage of typical hypertrophic obstructive cardiomyopathy.Most Appropriate Definition Taking the term "obstructive" as indic...

show abstract

Section: Unclassified Groupsupporting

confidence: 79%

Cardiac Function in Primary Myocardial Disorders--Part II

Goodwin¹

1964

BMJ

View full text Add to dashboard Cite

show abstract

“…Patient 5 was found to have only a left coronary artery, so flow is for the entire heart. This patient is a member of the family reported by Pare et al (8). The four normal patients had a mean ± 1 SE, left coronary artery plasma flow of 102 ± 13 ml/min, and a mean transit time of 19.2 ± 2.4 seconds.…”

Section: Resultsmentioning

confidence: 72%

“…Against this argument is our finding that blood flow as measured by 8 the water flow being higher than T-1824-calculated blood flow. Another potential error in calculating t for both indicators would be changes in coronary flow during the infusion period.…”

Section: Mean Transit Timementioning

confidence: 79%

“…The radiopaque material was removed from the catheter and replaced by heparinated saline. A constant infusion of indicator was then delivered by a velocity-regulated servo pump 8 at a rate of 3.82 ml/min for a period of 6 minutes. More rapid infusion rates tended to cause the catheter in the left coronary artery to be dislodged from the artery origin causing dye leak.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Blood Flow and Tissue Space of the Left Coronary Artery in Man

Klassen

Agarwal

Tanser

et al. 1970

Circulation Research

View full text Add to dashboard Cite

Using a constant infusion of two indicators, T-1824 bound to albumin and tritiated water, flow and transit time were measured in the left coronary system of intact man. Indicators were infused for 6 minutes into the left coronary artery with sampling from the coronary sinus in a region that drained exclusively the inflow of the left coronary artery and from the brachial artery for recirculation. The degree of heterogeneity of myocardial perfusion could be defined by the time required for the curve to reach a plateau. A correlation coefficient of 0.966 was found between the two indicator-measured blood flows. The average myocardial hematocrit was calculated and found to be similar to the arterial. In the presence of myocardial disease, total flow of the left coronary artery was increased. When this was divided by tissue volume, the blood flow per unit volume of tissue was decreased in the presence of the idiopathic cardiomyopathy.

show abstract

“…It is an example of inheritance through a dominant autosomal gene with a higher degree of penetrance (Bishop et al, 1962) of a condition described by Evans (1949) as familial cardiomegaly. The most remarkable example is the French-Canadian family starting in the seventeenth century that were described by Pare et al (1961). An account of another syndrome of cardiomyopathy, multiple flat pigmented naevi, dwarfism, and electroencephalographic changes is shortly to be published by Polani and Moynihan (personal communication): this syndrome is transmitted dominantly, either by a single gene with pleiotropic effect or by two closely linked genes.…”

Section: Genetic Causesmentioning

confidence: 99%

Causes of malformations of the heart.

Campbell¹

1965

BMJ

View full text Add to dashboard Cite

Hereditary cardiovascular dysplasia

Cited by 178 publications

References 37 publications

Cardiac Function in Primary Myocardial Disorders--Part II

Cardiac Function in Primary Myocardial Disorders--Part II

Blood Flow and Tissue Space of the Left Coronary Artery in Man

Causes of malformations of the heart.

Contact Info

Product

Resources

About