Mitral and aortic valve replacement in a patient with ectodermal anhydrotic dysplasia. A case report.

Ectodermal anhydrotic dysplasia is a rare, usually X-linked recessive malformation of ectodermal tissues and organs. The case of a 17-year-old boy with ectodermal anhydrotic dysplasia and concomitant combined mitral valve disease and aortic insufficiency is described. The surgical risk was considered to be high due to severe atrophia of the laryngo-pharyngeal mucosa complicating endotracheal intubation and the possibility of postoperative temperature control disturbances. Double valve replacement was performed late in the autumn, after laryngo-pharyngeal pharmacological treatment preparing for endotracheal intubation. The peri-operative course was managed without any complication related to the hereditary malformation. Our experience suggests that patients suffering from ectodermal anhydrotic dysplasia may undergo open heart surgery after appropriate preparation. Potential postoperative problems related to the hypohydrosis and impaired spontaneous temperature control could be prevented in our patient.

[Surgical treatment of bacterial endocarditis of the tricuspid valve]. / Bakteryjne zapalenie wsierdzia zastawki trójdzielnej leczone operacyjnie.

We present a rare case of bacterial endocarditis of tricuspid valve caused by temporary intracardiac pacing. The 48-year old male patient developed complete a-v block during the 1st day of acute inferior myocardial infarction. Intracardiac electrode was inserted for temporary pacing. After 4 days signs of bacterial endocarditis developed. Patient was markedly febrile, moderate tachycardia with gallop rhythm and systolic murmur of tricuspid valve insufficiency were present. Dullness to percussion was audible at the base of right lung. Hepato- and splenomegaly appeared during the second month of hospitalization. Laboratory tests revealed: elevated ESR, leukocytosis with a shift to the left, several blood cultures were positive to Staphylococcus aureus. On repeated chest X-ray patchy infiltrates with thin-walled translucent pools were visible. Transthoracic and transoesophageal++ echocardiography provided more precise informations. Bacterial vegetations were visualised on the tricuspid valve. Coronary angiography revealed proximal occlusion of the right coronary artery and 75-80% stenosis of the left circumflex artery. Antibacterial treatment guided by blood cultures was begun: vancomycin combined with netilmycin, then tienamycin and diflucan--after 10 weeks treatment was decided to be unsuccessful and the decision about surgical treatment was made. In extracorporeal circulation posterior left leaflet together with granular bacterial growths was excised. Septal and anterior leaflets were found normal. Cultures made of excised tissue were positive for Staphylococcus aureus and subsequent treatment with fluoroquinolones gave satisfactory result. Postoperative echocardiography revealed only small tricuspid valve insufficiency. Coronary by-pass surgery was performed later because of the high risk of simultaneous operation.(ABSTRACT TRUNCATED AT 250 WORDS)

[Concurrent coronary artery disease and acquired rheumatic mitral valve disease]. / Wspólistnienie choroby wiencowej z nabyta, poreumatyczna wada zastawki mitralnej.

The aim of this study was to estimate coincidence of coronary artery disease (CAD) and rheumatic mitral valve disease in 264 patients treated in the National Institute of Cardiology (1976-1990). Severity of stenoses on coronary angiography with respect to age, sex, symptoms and risk factors were also estimated. Stenoses over 70% of artery lumen in relation to artery diameter before lesion and over 50% for left main coronary artery were stated as severe. There were 180 (68%) females and 84 (32%) males in the studied group; mean age was 52.5 year. Patients were divided into two groups: with angina--126 pts and without CAD symptoms--138 pts. 8 females (4%) had severe stenoses and 45 (25%) non-severe. Respectively 14 males (16.7%) had severe stenoses and 14 non-severe. Severe lesions were present in a group of females older than 50 years and in a group of males older than 45 years. Both in group with or without angina prevalence of coronary artery lesions was similar. Sensitivity and specificity of CAD clinical symptoms was low (less than 50%). Significantly more risk factors were present in pts with coronary stenoses than in pts free of CAD. No correlation between high pulmonary artery pressure and angina in patients without coronary stenoses occurred.

[A problem with qualifying for heart surgery for a patient with severe congestive heart failure in both ventricles in the course of left aortic stenosis]. / Problem kwalifikacji do operacji kardiochirurgicznej chorego ze skrajna niewydolnoscia obukomorowa serca w przebiegu zwezenia lewego ujscia tetniczego.

A case of a patient 62 years old with a severe congestive heart failure in the course of aortic stenosis and after myocardial infarction was presented. A concomitant mitral insufficiency, tricuspid insufficiency and pulmonary hypertension was also observed. An excellent improvement after aortic valve replacement was reached.