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Objective: To describe aspects of the natural history and pathophysiology of giant low pressure pulmonary artery aneurysms and to propose potential surgical strategies.Design: Cross sectional retrospective review.Setting: Supraregional tertiary referral centre.Patients: All adult patients referred for assessment of giant pulmonary artery aneurysm retrospectively identified from the Mayo Adult Congenital Heart Disease Clinic database.Methods: Patient data were reviewed from hospital records, including echocardiograms, magnetic resonance images, radiographs, and histology slides.Results: Four patients were identified with a median age of 52 years (range 37-64 years). Presenting symptoms were effort related dyspnoea, chest discomfort, and hoarseness in one patient. All patients had pulmonary regurgitation and clinical evidence of right ventricular enlargement in association with a pulsatile mass at the upper left sternal edge. Transthoracic echocardiography showed the giant pulmonary artery aneurysm involving the main pulmonary artery and proximal branches, and confirmed severe pulmonary regurgitation in all patients. None of the patients had intimal tearing, medial dissection, or pulmonary arterial rupture. The pulmonary valve was replaced to relieve symptoms and preserve right ventricular function. Pulmonary arterial histology showed medial degeneration of elastic fibres with accumulation of basophilic ground substance.Conclusions: Rupture or dissection of these low pressure aneurysms is rare. The timing of surgical intervention should be determined by changes in right ventricular size and function resulting from pulmonary regurgitation or pulmonary stenosis, and not the size of the aneurysm. [ABSTRACT FROM AUTHOR]

Presents a study on the acute hemodynamic response of patients with chronic heart failure to pirbuterol. Information on the development of pirbuterol; Cardiovascular effects of oral pirbuterol; Comparisons between pirbuterol and other oral vasodilators.

A selected group of 18 patients aged 15-27 years with transposition of the great arteries and a previous Mustard procedure were evaluated to determine their functional ability and clinical state. Arrhythmias were common, occurring at some time in 16/18 (89%). Arrhythmia was serious in four; two of them required pacing and two had cardiac arrests, one resulting in death. Seven (41%) had right ventricular dysfunction; this was progressive in three. Tricuspid regurgitation was present in seven (41%); it occurred in patients with normal and reduced right ventricular ejection fractions. Regurgitation became progressively worse as the right ventricle dilated. Left ventricular function was well preserved in most patients. Fourteen (82%) of this pioneer group were leading normal lives (ability index 1 or 2). Although these results are acceptable concern remains about the probability of deteriorating right ventricular function. [ABSTRACT FROM PUBLISHER]

The outcome was reviewed in 17 patients aged 15-40 years with tricuspid atresia and transposed great arteries selected by survival beyond age 14 years. Only five lead normal lives (ability index 1 or 2); the rest are dead or disabled. Arrhythmias occurred in seven. Maintenance of sinus rhythm is important because incessant atrial arrhythmias cause serious symptomatic deterioration. Pulmonary vascular disease and subaortic stenosis were important determinants of late mortality and morbidity. Because the mortality associated with the Fontan operation was high in these patients it should be performed with impeccable surgical technique and only in those who fulfil all the selection criteria for the operation. A shunt is the preferred option when any of the criteria are not met. [ABSTRACT FROM PUBLISHER]

Twenty nine patients aged 15-35 years (mean 23) with tricuspid atresia and normally related great arteries were studied; 20 are alive. Ten patients who had had a Fontan operation (group 1) were compared with 10 patients with palliative shunts (9) or no surgery (1) (group 2). Patients were graded according to their ability to lead a normal life (ability index). Patients in group 1 tended to have a better ability index, a greater exercise capacity, and fewer social and extracardiac problems than those in group 2. The mean left ventricular ejection fraction measured by radionuclide angiography was the same in both groups. Arrhythmias were equally common in both groups; they appear to be age related and they occur independently of left ventricular function. [ABSTRACT FROM PUBLISHER]

Eleven patients with double mitral valve orifice and atrioventricular defects were studied, and the diagnosis proven by open heart surgery. The correct preoperative diagnosis was suggested by a characteristic angiographic appearance of the medial border of the left ventricle. M-mode echocardiography may show the two orifices which are better seen on two dimensional echocardiography. Two operative deaths occurred in patients with a slightly obstructive valve, and one late death followed mitral valve replacement. Eight patients did well after minor repair to a cleft, or leaving the valve untouched. Occasionally a double mitral orifice, if disorganised and slightly stenotic, may cause early deterioration in a patient with an otherwise uncomplicated ostium primum defect. The surgeon should be forewarned of this mitral anomaly since simple repair may be difficult. [ABSTRACT FROM PUBLISHER]

One hundred consecutive patients, admitted to the coronary care unit with cardiac pain at rest but no evidence of recent myocardial infarction have been followed up for nine to 26 (mean 14) months. They were treated initially with bed rest, beta-adrenergic blockade, and nitrates. In 54 patients pain subsided within 24 hours. Coronary angiography was carried out in 46. Thirty-five had coronary artery lesions and three had spasm in normal coronary arteries. One had hypertrophic cardiomyopathy and seven had normal findings. Seventeen patients with previous angina and severe coronary disease were operated on, with one death and one perioperative infarction; two died late, 12 were symptom free, and two had angina. Seven of 18 patients treated medically had recurrent angina and underwent operation. Of the 11 unoperated patients, one died, three had angina, and seven were symptom free. Two of the eight patients who were not catheterised developed infarction, four had angina, and three were symptom free. Recurrent pain continued for more than 24 hours in 46 patients, and all underwent angiography. Forty-three had coronary artery disease and 34 underwent early bypass surgery; there were two operative deaths and three perioperative infarctions. Twenty-six symptom free at follow-up. Of the nine unoperated patients with coronary disease, four developed infarction, two were operated on for recurrent angina, two were symptom free, and one had mild angina. Optimal management of patients with pain at rest can be determined only with knowledge of the coronary artery anatomy and of left ventricular function. Many respond initially to intensive medical treatment and coronary angiography can be performed electively. In those with continuing pain, urgent angiography is required and can be done safely. [ABSTRACT FROM PUBLISHER]

Two cases are described in which severe mechanical haemolytic anaemia developed shortly after operation for repair of non-rheumatic mitral regurgitation. One patient had a "floppy" valve and the other cleft mitral leaflets, and both had chordal rupture. In both there was residual regurgitation after repair though in one this was initially only trivial. Clinically manifest haemolysis ceased after replacement of the valve by a frame-mounted xenograft. There are two previously reported cases in which haemolytic anaemia followed an unsuccessful mitral valve repair operation. Subclinical haemolysis or mild haemolytic anaemia may occur with unoperated valve lesions, but hitherto frank haemolytic anaemia has been observed only when turbulent blood flow is associated with the presence of a prosthetic valve or patch of prosthetic fabric. In these four cases, however, polyester or Teflon sutures were the only foreign material, and it is suggested that when these are used for the repair of leaflets, particularly in non-rheumatic mitral valve disease, they may increase the damaging effect of turbulence on circulating red blood cells. [ABSTRACT FROM PUBLISHER]

Describes the case of 31-year-old woman diagnosed with left main coronary artery fistula exiting into the right atrium. Presenting signs and symptoms; Past medical history; Imaging procedures conducted; Treatment.