ABSTRACT
Ischemic cerebrovascular disease is an important cause of morbidity and mortality. One common final pathway in neuronal ischemic damage is the uncontrolled influx of calcium into the cell, mediated by voltage dependent channels or activation of the NMDA (N-methyl D-aspartate) receptor. The therapeutic utility of a non-competitive NMDA blocker (MK-801, 2 mg/kg i.p.), to prevent the neuronal ischemic damage in an experimental middle cerebral artery occlusion model has been tested. The drug was administered 10 minutes before (group 3) and one hour after the arterial occlusion (group 4), and the results were compared with a group in which no medicament was utilized (group 2) and a control group (sham operation, group 1). MK-801 reduced significantly the area of infarction in relation to the control group (p < 0.05), mainly if the MK-801 was administered before the occlusion (group 3). These results suggest that MK-801 may be useful for the prevention of the neuronal ischemic damage caused by focal ischemia. However, before recommending its use in humans, all the possible collateral effects must be defined.
Subject(s)
Brain Ischemia/drug therapy , Dizocilpine Maleate/therapeutic use , Receptors, N-Methyl-D-Aspartate/drug effects , Animals , Rats , Time FactorsABSTRACT
In 24 patients with aortic insufficiency undergoing aortic valve replacement, a clinical and hemodynamic study was performed pre-operatively. Left ventricular biopsies were obtained perioperatively for morphometric study. No significant relations were found when morphometric data were compared to functional class, cardiothoracic radio and ECG findings. The percentage of interstitial fibrosis was not correlated with any of the measured hemodynamic parameters. Myocardial cell diameter was weakly correlated with left ventricular systolic function parameters. A decrease in the percentage of contractile material was strongly correlated with an impaired left ventricular function, assessed pre-operatively. During clinical follow-up, patients were divided into two groups: Group A (17 patients) included patients who were in class I or II of NYHA after surgery. Group B (seven patients) included patients who died or were in functional class III or IV. As compared with Group A, Group B patients had a significantly lower ejection fraction; their myocardial cell diameter was larger and the percentage of myofibrils, and the content of contractile material were significantly lower. This suggests that, in aortic regurgitation, left ventricular dysfunction is correlated with contractile material loss and not with interstitial fibrosis, and that morphometric changes are good predictors of follow-up after surgery.
Subject(s)
Aortic Valve Insufficiency/physiopathology , Heart/physiopathology , Myocardium/pathology , Adolescent , Adult , Angiography , Aortic Valve/pathology , Aortic Valve/surgery , Aortic Valve Insufficiency/pathology , Aortic Valve Insufficiency/surgery , Child , Female , Follow-Up Studies , Hemodynamics , Humans , Male , Middle Aged , Myocardial Contraction , Myocardium/ultrastructure , Myofibrils/pathology , PrognosisABSTRACT
One case of traumatic aortic valve regurgitation operated upon is reported, and published cases of cardiac valve regurgitation caused by non-penetrating traumas are reviewed. This study demonstrates the relatively high frequency of traumatic heart diseases and among these, valve lesions. The dynamic disturbance occurs suddenly in a heart that is usually healthy and therefore without adaptation mechanism. This explains why such lesions are poorly tolerated and accounts for some peculiarities of semiology and haemodynamics. Plastic surgery seems to be successful in mitral valve lesions, whereas lesions of the aortic valve are such that valve replacement is required. The same applies to the tricuspid valve. The medico-legal aspects of the subject are mentioned.
Subject(s)
Aortic Valve Insufficiency/etiology , Thoracic Injuries/complications , Wounds, Nonpenetrating/complications , Accidents, Traffic , Adolescent , Aortic Valve/pathology , Aortic Valve/surgery , Aortic Valve Insufficiency/pathology , Aortic Valve Insufficiency/surgery , Heart Valve Prosthesis , Humans , Male , MotorcyclesSubject(s)
Heart Diseases/complications , Protein-Losing Enteropathies/etiology , Humans , Male , Middle AgedABSTRACT
Small diameter aortic valve bioprostheses are associated with resting ventriculo-aortic pressure gradients of 10 to 35 mmHg. In order to avoid this factor favouring degradation of left ventricular function and early deterioration of the bioprosthesis, we enlarged the aortic ring when the diameter was less than 23 mm in patients considered unsuitable for long-term anticoagulation. The surgical technique involved incising the annulus from the postero-lateral commissure to the anterior mitral leaflet and implanting a Dacron patch lined with pericardium. Nine patients aged from 10 to 70 years (average 22 years) underwent aortic valve replacement with a Carpentier-Edwards bioprosthesis associated with enlargement of the aortic ring, between June 1979 and December 1981. The mean follow-up period is now 18 months (range 9 to 39 months). One patient has been lost to follow-up. Before surgery, 6 patients were in Stage III and 3 patients in Stage IV of the NYHA classification. There were 4 patients with pure aortic regurgitation with valve prolapse, 1 patient with aortic regurgitation due to endocarditis, and 4 patients with mixed aortic valve disease. The underlying disease was rheumatic in 6 cases, congenital in 2 cases and infective endocarditis in 1 case. The mean diameter of the aortic ring before enlargement was 19 mm. After the procedure, it increased to 23,8 mm, so enabling the implantation of no 23 and no 25 bioprostheses. Three patients had associated mitral regurgitation, 3 patients had mixed mitral valve disease, 1 patient had a membranous VSD with infundibular stenosis, and 1 patient had subvalvular aortic stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Subject(s)
Bioprosthesis , Heart Valve Prosthesis , Adolescent , Adult , Aged , Aortic Valve/surgery , Aortic Valve Insufficiency/congenital , Aortic Valve Insufficiency/surgery , Child , Female , Follow-Up Studies , Humans , Male , Middle Aged , Rheumatic Heart Disease/surgeryABSTRACT
Between January, 1975, and January, 1982, 130 patients underwent mitral valvuloplasty for pure or predominant mitral insufficiency. Mean age at operation was 30 +/- 17 years. Twenty-five patients were under 15 years of age. Mitral insufficiency was mainly (112/130) due to rheumatic disease. Fifty-nine patients (45.4%) had another diseased valve which necessitated a surgical correction (tricuspid in 36 and aortic in 23). Surgical technique for mitral valvuloplasty varied according to the lesions. Three patients died in the first month after operation (2.3%). Five patients are lost to follow-up. The mean follow-up period for the 122 remaining patients is 38 +/- 27 months. Seven patients required reoperation and three of them died. An additional patient died without reoperation. Therefore, the late mortality was 3.1% (4/122). Almost all (116/118) of the remaining patients are in Class I (105) or II (11) of the New York Heart Association. Mean cardiothoracic ratio decreased from 60.6% +/- 7.7% preoperatively to 53.7% +/- 6.2% postoperatively (p less than 0.001). Thromboembolic episodes were noted in four patients, all of them in atrial fibrillation. Actuarial curves including hospital mortality showed a 92.0% survival rate at 7 years for the overall series (1.0% +/- 0.5%/patient-year), 93.7% +/- 4.9% at 7 years for isolated mitral reconstruction and 89.9% +/- 5.6% for mitral-tricuspid valvuloplasty at 5 years. The embolism-free rate at 7 years was 91.2%, with a rate of thromboembolic episodes of 1.0 +/- 0.5%/patient-year. Eighty-eight percent were free of reoperation at 7 years, with a rate of reoperation of 1.7 +/- 0.7%/patient-year. This system of mitral repair can provide stable functional results, low surgical and late mortality, and an acceptable rate of reoperation.
Subject(s)
Mitral Valve Insufficiency/surgery , Mitral Valve/surgery , Adolescent , Adult , Aged , Child , Female , Follow-Up Studies , Humans , Male , Methods , Middle Aged , Postoperative Complications/mortality , Recurrence , Reoperation , Rheumatic Heart Disease/etiology , Thromboembolism/etiologyABSTRACT
Takayashu's inflammatory panarteritis essentially results in stenotic lesions of the supra aortic axes and the collateral vessels of the abdominal aorta. Fusiform, segmental post-stenotic dilatation is commonly observed. Three particular types of aneurysms are described, each illustrated by a case report: --aneurysm of the ascending aorta causing severe aortic regurgitation by dilatation of the aortic ring: the diagnosis of Takayashu's disease was made in a young West Indian female on the evidence of associated aortic lesions and calcification of the descending thoracic aorta. The patient underwent replacement of the ascending aorta and aortic valve replacement. This form is very rare, although mild aortic regurgitation in Takayashu's disease has been reported in about ten cases by different workers; --large saccular aortic aneurysm. This lesion of the descending thoracic aorta with parietal calcification and without intrasaccular thrombosis was associated with a long, irregular stenosis of the paroxismal segment of the aorta, giving rise to signs of coarctation, and with multiple stenoses of the supra-aortic axes in a 31 year old Algerian. Surgical cure was realised by occluding the orifice in order to avoid replacing the aorta at the origin of the main medullary arteries. An ascending aorta-abdominal aorta bypass was performed at the same time, together with correction of the stenoses of the supra-aortic vessels. A few similar cases of large succular aneurysms of the thoracic or abdominal aorta or of the large collateral vessels have previously been described; --pseudo-aneurysm due to arterial rupture and formation of a large hematoma. A 20 year old Algerian presented with a polylobulated saccular aneurysm of the superficial femoral artery in the femoral triangle. Several stenotic lesions typical of Takayashu's disease (including a particularly rare stenosis of the contra lateral superficial femoral artery) were associated. At surgery, the lesion was found to be a pseudo-aneurysm with no true arterial wall on histological examination. The role of an infective endarteritis is discussed and excluded. This type of lesion does not appear to have been previously reported. These three varieties of aneurysm were the presenting signs of Takayashu's disease in the cases reported.
Subject(s)
Aortic Aneurysm/etiology , Aortic Arch Syndromes/complications , Takayasu Arteritis/complications , Adult , Aorta, Thoracic/diagnostic imaging , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/pathology , Female , Humans , Male , RadiographyABSTRACT
A haemodynamic and cineangiographic study was conducted in 20 patients with chronic aortic regurgitation alone or associated with mitral regurgitation before and during i.v. administration of isosorbide dinitrate 5 mg/hour. Freedom from coronary disease had been ascertained. The heart rate and aortic pressure (initially normal), cardiac index (initially low), pulmonary pressures and pulmonary and systemic resistances (slightly raised initially) remained unchanged. On the other hand, the left ventricular (LV) filling pressure, distinctly raised before treatment, was reduced by 17% (p less than 0.05). There was also a 10% reduction in LV end-diastolic volume (from 204 +/- 60- cm3.m2 to 184 +/- 56 cm3,m2; p less than 0.001) and a 14% reduction in LV end-systolic volume (from 104 +/- 39 cm3.m2 to 89 +/- 40 cm3.m3; p less than 0.001). LV geometry, stroke volume and regurgitation volume were unmodified. There was a significant improvement in ventricular function indices, globally reduced before treatment: + 8% for the fiber shortening amplitude (p less than 0.025), + 6% for the ejection fraction (p fiber shortening (p less than 0.01), and + 15% for the ESP: ESV ratio (p less than 0.05). The passive elasticity indices, all increased before treatment, also improved. It is concluded that isosorbide dinitrate improves LV systolic and diastolic functions in patients with chronic valve disease.
Subject(s)
Aortic Valve Insufficiency/drug therapy , Isosorbide Dinitrate/pharmacology , Mitral Valve Insufficiency/drug therapy , Mitral Valve Stenosis/drug therapy , Adolescent , Adult , Aged , Elasticity , Female , Heart Ventricles/drug effects , Hemodynamics/drug effects , Humans , Male , Middle Aged , Systole/drug effectsABSTRACT
Hemodynamic studies were performed in 26 patients 2-60 months after mitral valvuloplasty (average 14 months). All patients but one had an annular deformity requiring prosthetic ring annuloplasty. In addition, 25 patients had prolapsed leaflet or restricted leaflet motion requiring specific techniques of repair. All patients were asymptomatic after operation. Significant residual mitral insufficiency was present in only five patients. Roentgenograms showed a reduction in cardiothoracic ratio, from 0.62 +/- 0.07 preoperatively to 0.53 +/- 0.06 postoperatively (p less than 0.001). Mean pulmonary artery pressure decreased from 30 +/- 12 mm Hg to 18 +/- 6 mm Hg (p less than 0.001) and cardiac index returned to normal (2.7 +/- 0.7 1/min/m2 vs 3.1 +/- 0.7 1/min/m2, p less than 0.02). Angiocardiographic studies showed that end-diastolic volume index returned to normal (148 +/- 41 cm3/m2 vs 89 +/- 23 cm3/m2, p less than 0.001), with significant decreases in end-systolic volume index ( 63 +/- 27 cm3/m2 vs 43 +/- 16 cm3/m2, p less than 0.001), left ventricular mass (114 +/- 28 g/m2 vs 88 +/- 20 g/m2, p less than 0.001), ejection fraction (58 +/- 11% vs 52 +/- 11%, p less than 0.02) and mean velocity of fiber shortening (1.19 +/- 0.36 circ/sec vs 1.01 +/- 0.24 circ/sec, p less than 0.05). We conclude that mitral valvuloplasty provides good and stable midterm results and that the operation should be performed before irreversible myocardial lesions occur.
Subject(s)
Cineangiography , Hemodynamics , Mitral Valve Insufficiency/surgery , Mitral Valve/surgery , Adolescent , Adult , Child , Female , Humans , Male , Methods , Middle AgedABSTRACT
Twenty patients with pure or prevalent mitral regurgitation, undergone mitral valve conservative surgery according to the Carpenter-technique from 1975 to 1979, have been studied. There are 19 women and 1 man, mean age of 25 years (range 11 to 65); 18 had a rheumatic fever; 2 had a mitral valve prolapse due to a degeneration of collagen within the central core of the cordae tendineae, which was the primarily responsible for cordae rupture in 1 case. The clinical and hemodynamic features were severe in a 50 per cent of cases (8 patients in N.Y.H.A. functional class III; 3 in class IV); there was pure mitral regurgitation in 12, combined mitral stenosis and regurgitation in 8; tricuspidalic regurgitation which needed annuloplasty in 5 patients. All the patients have been studied by hemodynamic and angiocardiographic study before and an average of 14 months after surgical treatment. Surgical conservative technique of mitral valve is described; the results are analyzed. All the patients are in N.Y.H.A. functional class I, 14 months after surgical valve therapy. In three cases, a systolic 3/6 murmur which was present immediately after surgical treatment and that not increased in time, remains. EKG left overload is still present in 2 cases; Heart-Chest ratio decreases in all the patients (range 0,61 to 0,51). Atrial fibrillation is present in 6 patients. Hemodynamic findings show: Pulmonary Systolic Pressure (PSP) decreases (45 +/- 4 to 27 +/- 1); Mean Pulmonary Capillary Wedge (PCW) pressure decreases (19 +/- 1 to 11 +/- 1); Total Peripheral Resistance (TRP) (2023 +/- 112 to 1595 +/- 70 dynes sec cm--5), Total Pulmonary Resistances (TPR) (742 +/- 89 to 351 +/- 36), Pulmonary Arterial Resistances (PArtR) (344 +/- 51 to 133 +/- 18 dynes sec cm--5), and Left Ventricle Diastolic Pressure (LVEDP) (12 +/- 1 to 8 +/- 1 mmHg) normalize. The Ejection Fraction (EF) decreases at the most operative control after surgery in 11 patients (56 +/- 3 to 50 +/- 1). Angiographic study shows no regurgitation in 4 patients, a least regurgitation in 13 cases and there is a 2/4 regurgitation in 3 cases only. The AA. have got to an excellent result in 17 patients; a 2/4 degree angiographic regurgitation persist in 3 cases, although a clear clinical improvement. The AA. consider of great utility this surgical valvular management according to a critical review of the efficacy and stability of the results.
Subject(s)
Cineangiography , Hemodynamics , Mitral Valve/surgery , Adolescent , Adult , Aged , Child , Electrocardiography , Female , Heart Ventricles/physiopathology , Humans , Male , Middle Aged , Mitral Valve/diagnostic imaging , Mitral Valve Insufficiency/complications , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/surgery , Mitral Valve Prolapse/surgery , Postoperative Care , Preoperative Care , Rheumatic Fever/complications , Stroke VolumeABSTRACT
Left ventricular myocardial biopsy was carried out at operation in 11 patients with pure, isolated mitral stenosis after preoperative angiocardiographical assessment of left ventricular function. The biopsy specimens were examined by light and electron microscopy. The diameter of the myocytes was normal (20 +/- 1.6 mu). The changes observed were probably of a degenerative type with anarchy and irregularities in the sarcomeres, modification of the Z bands, as seen in Nemaline myopathies, and changes in the intercalatory discs. Moderate interstitial fibrosis with scanty histiocytes was also observed. A quantitative assessment by two dimensional planimetry showed a significant increase in the interstitial space (37 +/- 5.5%) compared to a control group without fibrosis (22 +/- 1.1%). The angiocardiographical indices of left ventricular function were all decreased. The amplitude of circumferential fibre shortening was reduced: 25 +/- 6% the ejection fraction by 52 +/- 9% and the average speed of circumferential fibre shortening by 1.0 +/- 0.3 circ/s. Only four patients had normal left ventricular function (ejection fraction > 55%). However, it was not possible to establish a significant correlation between the degree of fibrosis and the reduction in left ventricular function. Left ventricular fibrosis may be one of the factors responsible for the reduction of myocardial function, but it does not in itself explain all the changes in left ventricular function observed in mitral stenosis.
Subject(s)
Angiocardiography , Mitral Valve Stenosis/pathology , Myocardium/pathology , Adolescent , Adult , Female , Heart Ventricles/diagnostic imaging , Heart Ventricles/pathology , Heart Ventricles/ultrastructure , Hemodynamics , Humans , Male , Mitral Valve Stenosis/diagnostic imaging , Myocardium/ultrastructureABSTRACT
Left ventricular myocardial biopsies were performed during surgery in 11 patients with pure and isolated mitral stenosis. Patients had undergone a preoperative angiocardiographic study of left ventricular function. Biopsy specimens were examined with the light and electron microscope. Myocyte cell diameter was normal (20 +/- 1.6 mu). Lesions existed which were probably degenerative, including anarchy and irregularities of sarcomeres, Nemaline Myopathy-type Z line changes and alterations of intercalated discs. A moderate fibrosis was found in the interstitial spaces with very few histiocytes. The coincidence planimetry study of the interstitial spaces showed a 37 +/- 5.5% increase compared to a control group with no fibrosis (23 +/- 1.5%, p less than 0.01). The angiocardiographic indices of left ventricular function were all decreased. Only four subjects had normal left ventricular function (EF greater than or equal to 55%). Nevertheless, it was not possible to establish a significant correlation between the extent of fibrosis and the decrease of left ventricular function. Although left ventricular fibrosis could be one of the factors responsible for decreased myocardial function, it is not sufficient to explain the changes of left ventricular function which are rather frequently observed in mitral stenosis.
Subject(s)
Mitral Valve Stenosis/pathology , Myocardium/ultrastructure , Adolescent , Adult , Female , Heart Ventricles/diagnostic imaging , Heart Ventricles/pathology , Heart Ventricles/ultrastructure , Hemodynamics , Humans , Male , Myocardium/pathology , RadiographyABSTRACT
Lobstein's disease, a hereditary disorder of connective tissue, may rarely be accompanied by aortic or mitral regurgitation due to valvular dystrophy. The case reported is that of a patient with severe aortic incompetence with dilatation of the ascending aorta, complicated by angina pectoris and left ventricular failure; the patient also had Lobstein's disease with numerous spontaneous fractures, transverse lines of ossification, blue sclera skeletal deformities, and a positive family history of blue sclera. At surgery, valvular dystrophy was confirmed and corrected by aortic valve replacement. The patient's brother died recently with the same pathological association of Lobstein's disease and aortic incompetence. 28 cases of valvular disease and Lobstein's disease have been reported 12 with pathological evidence (at operation) on the purely dystrophic origin of the valvular regurgitation, and 2 with histological diagnoses alone in two newly born children. Aortic incompetence with dilatation of the ascending aorta is commoner than mitral incompetence. The macroscopical and histological appearances are similar to Marfan's syndrome and account for the operative risk of valvular replacement and for the incidence of postoperative haemorrhage. The rarity of valvular dysfunction in Lobstein disease contrasts with its relatively common occurrence in Marfan's syndrome.
Subject(s)
Aortic Valve Insufficiency/complications , Osteogenesis Imperfecta/complications , Adult , Aortic Valve Insufficiency/diagnosis , Aortic Valve Insufficiency/surgery , Female , Humans , Male , Middle AgedSubject(s)
Cineangiography , Hemodynamics , Mitral Valve Stenosis/physiopathology , Adolescent , Adult , Aged , Aging , Blood Pressure , Cardiac Output , Female , Heart Ventricles/physiopathology , Humans , Hypertension, Pulmonary/pathology , Male , Middle Aged , Myocardial Contraction , Papillary Muscles/pathologySubject(s)
Aortic Valve Insufficiency/physiopathology , Heart Valve Prosthesis , Hemodynamics , Myocardial Contraction , Adult , Angiocardiography , Aortic Valve Insufficiency/surgery , Blood Pressure , Cardiac Catheterization , Cardiac Output , Cineangiography , Diastole , Female , Humans , Male , Middle Aged , Postoperative Period , Pulmonary Circulation , Stroke Volume , Systole , Vascular ResistanceABSTRACT
A comparative study of the diagnosis of intracardiac left-to-right shunts by isotope radiography on the one hand and oxymetry, abnormal catheter trajectory and angiocardiography on the other, was performed on a series of 110 patients, comprising 12 normals, 46 valvulopathies or cardiomyopathies without shunts, 23 ASDs, 22 VSDs, and 7 other shunts. Intravenous injection of Technetium 99 m with scintigraphic imagery and interpretation of time-activity curves detected even small left-to-right shunts and appeared to be more sensitive than oxumetry. The estimation of the size of the shunt correlated well with oxymetry for VSDs but not for ASDs. The localisation of the shunt was more difficult. The atrial level of the defect was detected in some but not the majority of cases. The isotopic technique appeared unreliable in the presence of severe haemodynamic disturbances. The isotopic method is a quick means of detecting left-to-right shunts without measurable risk, particularly useful in the investigation of children and young adults thought to have left-to-right shunts.
Subject(s)
Heart Defects, Congenital/diagnostic imaging , Technetium , Adolescent , Adult , Aged , Cardiac Catheterization , Cardiomyopathies/physiopathology , Child , Child, Preschool , Heart Valve Diseases/physiopathology , Humans , Injections, Intravenous , Middle Aged , Radionuclide ImagingABSTRACT
The cardiac complications of radiotherapy of the thorax (e.g. for Hodgkin's disease and carcinoma of the breast) are various: 1) Pleural effusions, occurring a few months to two years after radiotherapy, spontaneously curable but may give signs of tamponnade or lead to constriction. 2) Constrictive pericarditis often several years after irradiation, with a poor prognosis in spite of attempts at pericardectomy which is indicated at the stage of hemodynamic constriction. 3) Myocardial involvement with advanced fibrosis and with various clinical presentations. The failure of pericardectomy and mitral incompetence were evidence of myocarditis in the case reported here.
Subject(s)
Mitral Valve Insufficiency/etiology , Myocarditis/etiology , Pericarditis, Constrictive/etiology , Radiotherapy/adverse effects , Adolescent , Female , Hodgkin Disease/radiotherapy , Humans , Pericarditis, Constrictive/surgery , Pleural Effusion/etiologyABSTRACT
The case is reported of carcinoid heart disease in a lady of 70 with intractable congestive cardiac failure 5 years after the removal of a primary carcinoid tumour of the ovary. The special features of primary carcinoid tumours of the ovary are recalled, with emphasis on their rarity and of the absence of liver metastases. The various features of carcinoid syndrome are recalled in the light of current knowledge of the pathogenesis. A review of the literature on cardiac involvement in primary carcinoid tumours of the ovary, amounting to 10 cases, is included. The possibility of surgical cure of the heart lesions in carcinoid tumour by a prosthetic tricuspid valve are discussed, in the light of the 6 reported cases and the present one. Our report is the first one of replacement of the valve after removal of a primary ovarien carcinoid tumour, and the excellent result has been maintained after three years.
Subject(s)
Carcinoid Tumor/complications , Heart Valve Prosthesis , Ovarian Neoplasms/complications , Tricuspid Valve Insufficiency/surgery , Aged , Female , Follow-Up Studies , Humans , Tricuspid Valve Insufficiency/etiologyABSTRACT
A case is reported of hypoplasie of the right ventricle with a wide atrial septal defect in a child of 10. The presence of cyanosis with right atrial and left ventricular preponderance led us to suspect tricuspid atresia, but this was excluded by catheterisation and angiocardiography. There was definitely and atrial septal defect with a two-directional shunt, but the tricuspid valve could be negotiated, and the right-sided opacity provided the essential diagnostic criterion, namely hypoplasia of the sinus portion of the right ventricle with a normal infundibulum. 17 other published cases of hypoplasia of the right ventricle with atrial septal defect are reviewed. The current indications for surgery are discussed in the light of the nature of the disordered physiology. Simple closure of the atrial septal defect, which was carried out successfully in 3 cases, appears to be of great value in the larger child, when there is a wide defect.