ABSTRACT
We report a case of Williams and Beuren syndrome in which the obstruction of the pulmonary vascular bed, linked with peripheral stenoses of the pulmonary arteries, associated here with an interventricular communication, was aggravated due to the coexistence of in situ thrombotic disease, leading to a progressive hypoxaemia, requiring long-term anticoagulant treatment, and compromising the long-term prognosis. This case report puts into question the so-called favourable prognosis of pulmonary arterial branch stenoses in this malformation syndrome.
Subject(s)
Arterial Occlusive Diseases/diagnostic imaging , Pulmonary Artery/pathology , Williams Syndrome/complications , Heart Ventricles/diagnostic imaging , Humans , Infant , Male , Pulmonary Artery/diagnostic imaging , Tomography, X-Ray ComputedABSTRACT
We report the case of a 68 years old patient with platypnea orthodeoxia syndrome who has been clinically suspected on cutaneous saturation position's variation. It has been confirmed by transthoracic and transesophageal echocardiography (TEE). TEE showed the size of patent foramen ovale (PFO), visualised the right to left shunt. A right heart angiography confirmed echocardiographic data and allowed successful closure by a Cardioseal percutaneous transcatheter button device implantation. Symptom's disappearance and a TEE control after 2 months confirmed the success of the procedure.
Subject(s)
Heart Septal Defects, Atrial/diagnosis , Hypoxia/etiology , Aged , Cardiac Catheterization , Diagnosis, Differential , Echocardiography , Echocardiography, Transesophageal , Female , Heart Septal Defects, Atrial/surgery , Humans , SyndromeABSTRACT
A large fistula between the left circumflex coronary artery and the distal branches of the right pulmonary artery resulting in myocardial ischemia in a 52 year old woman with no angiographic atheromatous coronary lesions, was occluded by a detachable balloon introduced percutaneously and positioned at the origin of the fistula. A control angiogram performed one month later showed the balloon in position and the disappearance of the fistula. The Thallium myocardial scintigraphic abnormalities regressed after occlusion of the fistula. This is the second case (the first one in France) of radical non-surgical treatment of a congenital coronary fistula.
Subject(s)
Arterio-Arterial Fistula/therapy , Catheterization/methods , Coronary Vessels , Pulmonary Artery , Angiocardiography , Arterio-Arterial Fistula/complications , Arterio-Arterial Fistula/congenital , Coronary Angiography , Coronary Disease/etiology , Coronary Disease/therapy , Female , Humans , Middle AgedABSTRACT
The modified Fontan procedure is being used in an increasing number of complex cyanotic cardiac lesions with pulmonary stenosis. Seven patients aged 11 to 24 years (average 17.5 years) underwent surgery by a technique derived from the Fontan procedure: direct atriopulmonary anastomosis without a tube or valve. The tricuspid valve when patent was closed with a patch. The diagnoses were: single ventricule (4 cases), Taussig-Bing anomaly (2 cases) and tricuspid atresia (1 case). All patients had associated pulmonary stenosis with low pulmonary vascular resistances. The great vessels were in L-malposition in 3 cases. The hospital mortality was nil. Transient atrial fibrillation was observed in 2 cases and was well tolerated clinically. The follow-up period ranges from 2 months to 4 years (average 2.3 years). All patients are acyanotic with no signs of right-sided failure and in sinus rhythm. Control cardiac catheterisation and angiography were performed in 6 cases and showed good function of the anastomosis and a mean atrial pressure of 14 mmHg. Direct atriopulmonary anastomosis offers a very acceptable surgical solution to certain forms of single ventricle or equivalent with low pulmonary pressures. The short and medium term results seem to be better than those of intraventricular repair.
Subject(s)
Heart Atria/surgery , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Pulmonary Artery/surgery , Adolescent , Adult , Child , Humans , Methods , Postoperative Complications , Time Factors , Transposition of Great Vessels/surgery , Tricuspid Valve/abnormalitiesABSTRACT
The authors reports the detection of a rare and remarkably well tolerated cardiac malformation in a 40 year old woman whose clinical presentation would have suggested an ordinary mitral stenosis, had there not been a number of very unusual signs. Cardiac catheterisation and angiography showed a corrected transposition of the great arteries with stenosis of the systemic atrioventricular valve. This case is used to illustrate the principal malformations observed in association with corrected transposition of the great arteries, especially the common and very variable abnormalities of the systemic atrioventricular valve. The unusual feature of the reported case was the stenotic nature of the valvular lesion and the fact that this was the only associated intracardiac abnormality, simulating, at first sight, a rheumatic mitral stenosis.
Subject(s)
Mitral Valve Stenosis/diagnosis , Transposition of Great Vessels/diagnosis , Adult , Angiography , Electrocardiography , Female , Humans , Mitral Valve Stenosis/diagnostic imaging , Mitral Valve Stenosis/surgery , Transposition of Great Vessels/diagnostic imaging , Transposition of Great Vessels/surgeryABSTRACT
Between April 1979 and September 1981, 98 patients have undergone valve replacement with an Ionescu-Shiley bovine pericardium xenograft (54 men and 44 women, average age 53 years, range 13 to 76 years). Fifty-three patients underwent aortic valve replacement (AVR); 28 had a mitral valve replacement (MVR) and 17 had double valve replacement (MAVR). Nine patients (9%) were operated as an emergency for endocarditis or for acute malfunction of a previously implanted prosthesis. The hospital mortality was 9% (AVR: 7%, MVR: 7%, MAVR: 17%). The eighty nine survivors were followed up for 6 to 37 months (average: 21 months). All patients were examined or contacted directly (as were the treating cardiologists) during the 2nd trimester of 1982. Eight patients were lost to follow-up. Six patients died during follow-up (6,7%) 3 to 22 months after surgery. The valvular prosthesis was the cause of death in 2 cases. Although only 37 patients (41%) were maintained on long-term anticoagulant therapy, there were no cases of thrombosis of the valve and the incidence of embolic complications was 0,9% per patient year in AVR and 2,6% per patient year in MVR. Four patients (2,6% per patient year) developed endocarditis on their prosthesis and 3 were cured by medical treatment alone. One patient was reoperated 1 year after the initial operation. One case (0,7% per patient year or 0,5% per valve year) of premature calcification was observed (18th month) in a 16 year old boy who was reoperated as an emergency without any success.(ABSTRACT TRUNCATED AT 250 WORDS)
Subject(s)
Bioprosthesis , Heart Valve Prosthesis , Adolescent , Adult , Aged , Aortic Valve/surgery , Bioprosthesis/adverse effects , Echocardiography , Endocarditis, Bacterial/etiology , Female , Follow-Up Studies , Heart Valve Diseases/surgery , Heart Valve Prosthesis/adverse effects , Hemodynamics , Humans , Male , Middle Aged , Mitral Valve/surgery , Thromboembolism/etiologyABSTRACT
Chronic right ventricular failure was observed in a young female operated at 25 years of age for Fallot's tetralogy despite surgical refixation of the patch closing the ventricular septal defect which had previously worked loose. The signs of right ventricular failure were due to severe tricuspid incompetence, confirmed at catheterisation and selective right ventricular angiography. They were associated with major hypoproteinaemia which was not due to urinary loss nor hepatic dysfunction, but which was attributed to an exudative enteropathy. The correction of the valvular defect by valvuloplasty was followed by rapid correction of the biological abnormality. The authors review the relationship between serum proteins and cardiac disease, discuss those cardiac affections with known associations with exudative enteropathy, and also the possibilities of reversing the biological abnormality after surgical cure of the causal cardiac lesion.
Subject(s)
Hypoproteinemia/etiology , Postoperative Complications/etiology , Tetralogy of Fallot/surgery , Tricuspid Valve Insufficiency/etiology , Adult , Female , Heart Valve Prosthesis , Humans , Tricuspid Valve/surgery , Tricuspid Valve Insufficiency/surgerySubject(s)
Cardiomyopathy, Hypertrophic/etiology , Heart Defects, Congenital/complications , Acute Disease , Cardiac Catheterization , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/surgery , Echocardiography , Female , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Hemodynamics , Humans , Infant , Male , Retrospective StudiesABSTRACT
The authors describe the unusual cardiac malformation in a young girl with Turner's syndrome: right ventricular hypoplasia and atrial septal defect, resulting in central cyanosis. A trial of temporary occlusion of the atrial septal defect at cardiac catheterisation before surgical closure suppressed the right-to-left shunt and the cyanosis without significantly changing the hemodynamic parameters. The authors review the types of cardiovascular abnormalities associated with Turner's syndrome and emphasize the value of a temporary occlusion test of defects in certain cyanotic cardiac malformations with atrial right-to-left shunts; it may lead to a relatively simple surgical procedure, as in the case described.
Subject(s)
Cardiac Catheterization , Cyanosis/etiology , Turner Syndrome/complications , Adult , Cyanosis/diagnosis , Cyanosis/surgery , Female , Heart Septal Defects, Atrial/complications , Heart Ventricles/abnormalities , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Humans , Radiography , Turner Syndrome/diagnosis , Turner Syndrome/surgeryABSTRACT
Among the primary myocardiopathies resulting from a known cause, acute infectious myocarditis plays an important part. This condition, usually interstitial myocarditis of viral etiology, occurs predominantly in the infant under two years of age. They are characterized by a hypotrophic hypokinetic myocardiopathy which is very hard to differentiate from endocardial fibroelastosis. Fibroelastosis is probably only one possible result of the course of certain myocarditis'. However, on the whole, prognosis is good and the children recover. Typhoid fever and diphtheria may also compromise myocardial function.
Subject(s)
Infections/complications , Myocarditis/etiology , Acute Disease , Bacterial Infections/complications , Child , Child, Preschool , Diagnosis, Differential , Echocardiography , Humans , Infant , Infections/diagnosis , Infections/drug therapy , Myocarditis/diagnosis , Myocarditis/drug therapy , Time FactorsABSTRACT
All cardiovascular malformations compromise ventricular function and lead to alterations in the myocardial structure. Most of these are adaptive reactions. However, in some cases, this reaction overreaches its object and leads to an authentic myocardiopathy. This myocardiopathy is usually hypertrophic in obstacles which result in systolic overload of the left ventricle (coarctation peri-orificial stenosis of the aorta, systemic hypertension) or of the right ventricle (pulmonic stenosis), or congestive in diastolic overload by left-to-right shunts or valvular insufficiency. These myocardiopathies may prevent recognition of the signs of the underlying heart disease thereby leading to diagnostic errors and delay in therapy. They may constitute the main factor of an operative decision.