Anomalies of the coronary arteries in children.

BACKGROUND: Anomalies of the coronary arteries in children occur very rarely. Congenital coronary anomalies may be isolated, or they may accompany other congenital heart defects, such as Fallot's Syndrome, transposition of the great arteries, or pulmonary atresia. The most common cause of acquired abnormalities of the coronary vessels in children is Kawasaki Disease. The purpose of the present study is to analyze the clinical picture and diagnostic methodology for coronary anomalies in children. MATERIAL AND METHODS: In 62,320 children a routine echocardiographic assessment was made of the ostium and the course of the initial segment of the coronary arteries. Anomalies of the coronary arteries were found in 29 children, ranging in age from 1 day to 12 years. In 25 of these cases the diagnosis was established on the basis of two-dimensional echocardiography with Doppler flow mapping, confirmed in 16 cases by aortography. In 3 children with Fallot's Syndrome and in 1 child with common arterial trunk, the coronary anomalies were found in angiocardiography. RESULTS: Isolated abnormalities of the coronary vessels were found in 18 children, including 13 cases of anomalous left coronary artery from the pulmonary artery, 1 case of anomalous right coronary artery from the pulmonary artery, and four cases of coronary fistula. In 9 children anomalies of the coronary arteries accompanied other congenital heart defects: 4 children with Fallot's Syndrome were found to have left descending coronary artery arising from the right coronary artery; 1 child with a common arterial trunk was diagnosed with anomalous common origin of coronary arteries; 3 children with transposition of the great arteries were found to have coronary anomalies (including 2 cases of anomalous circumflex branch from the right coronary artery, and 1 case of intramural course of the left coronary artery), and one child with pulmonary atresia was found to have coronary fistulas. Of the 2 children with Kawasaki Disease, one was diagnosed with an aneurysm in the left coronary artery, and the other with dilatation of the coronary arteries. The infants with abnormal divergence of the left coronary artery showed clinical and electrocardiographic signs of myocardial ischemia, while the newborns with fistulas presented with symptoms of circulatory insufficiency. These children required emergency surgical treatment. CONCLUSIONS: The clinical picture of children with anomalies of the coronary vessels may be heterogeneous, ranging from a lack of symptoms to very acute, even life-threatening symptoms. Infants with anomalous left coronary artery from the pulmonary artery and with hemodynamically significant fistulae require emergency surgical treatment. Echocardiographic examination enables the diagnosis of anomalies of the coronary arteries. In the majority of cases an aortography test is necessary before the patient is referred for surgery.

[Mitral valve prolapse in children]. / Wypadanie platka zastawki dwudzielnej u dzieci.

Mitral valve prolapse (MVP) is an abnormal movement of one or two of mitral leaflets into the left atrium during ventricular systole. Etiopathogenesis is still not fully understood. Clinical presentation of MVP could be asymptomatic. Chest pain, palpitation and syncope are typical for mitral valve prolapse syndrome. On physical examination most patients have midsystolic click and/or systolic murmur which is often misdiagnosed as an innocent murmur. Arrhythmia and mitral regurgitation are the most often seen complications of MVP and their number increases with patients age. Most children with MVP do not need any treatment. In those with mitral regurgitation prophylaxis against endocarditis is obligatory. The use of echocardiography increased the number of patients with diagnosed mitral valve prolapse diagnosed.

[Arrhythmia and repolarization in children with mitral valve prolapse]. / Zaburzenia rytmu serca i repolaryzacji u dzieci z wypadaniem platka zastawki dwudzielnej.

The aim of our study was to analyse arrhythmia and ST-T abnormality in patients with mitral valve prolapse (MVP). Study included 78 children, 53 girls and 25 boys aged between 4 and 17 years. In all children 12 lead ECG and 24 hours ECG Holter monitoring were performed with special concern to QT and QTc analyses. Patients were divided into two groups: group I included 13 pts with MVP without any mitral regurgitation (MR) and group II included 65 pts with MR. In group I 6 (47%) pts had mild arrhythmia. In group II 40 pts (62%) had different arrhythmias, among them 29 pts had mild arrhythmia (supraventricular and ventricular premature ectopic beats, atrio-ventricular block grade I) and 11 had severe arrhythmia (pair of ventricular ectopic beats, supraventricular tachycardia, ventricular tachycardia, atrio-ventricular block grade II). Thirteen pts with mild arrhythmia had chest pain, 17 had mild (< I grade) MR and 12 had severe (II-IV grade). In pts with severe arrhythmia 9 had symptoms, 8 had mild MR and 3 had severe MR (II-III degrees). Two (15%) pts from group I and 20 (31%) pts from group II had ST-T abnormalities. One patient (Syn Marfana) had long QTc.

Echocardiographic assessment of right atrial thrombus related to the implanted port device in patient receiving chemotherapy for non-Hodgkin's lymphoma.

The case of a potentially life-threatening complication related to the use of implanted port device in a 8 year old Non-Hodgkin's Lymphoma patient receiving chemotherapy is described. The device was inserted in early 1997 and used repeatedly for chemotherapy without any complications. In late 1997 during routine screening for cardiac left ventricular function before re-introduction of chemotherapy, an abnormal 1.43 x 1.53 cm mass, consistent with a non-mobile thrombus was found in the right atrium. The initial thrombolytic therapy with recombinant tissue plasmin activator (rt-PA) infused by a central venous catheter was combined with daily echocardiographic examination in order to assess both the timing and mode of thrombus resolution. After 8 days systemic fibrinolytic therapy was discontinued as major hemorrhage from venipuncture sites occurred and the clot dissolution was not obtained. Patient underwent right atriotomy utilizing cardiopulmonary bypass and subsequent surgical thrombus removal was successful. The study evaluated the contribution of two-dimensional echocardiography (2D) in the follow-up of vascuport and other central venous catheter (CVC) location and early diagnosis of related complications such as thrombi. The authors consider that pulmonary flow analyzed with Doppler echocardiography as a reliable, suitable and non-invasive method to evaluate increased pulmonary artery pressure in children with right atrial thrombi and probability of pulmonary microembolism or embolism. As the incidence of right atrial thrombi is highly associated with the catheter tip position in the right atrium, in contrast to their positioning in the superior vena cava or in its junction with the right atrium, the authors recommend that special attention and effort should be given to placing of the catheter tip in the superior vena cava or in its junction with the right atrium avoiding the right atrium during the implantation procedure. The surgical right atrium thrombus removal in patients with no clot dissolution despite systemic thrombolytic treatment underscores the importance of surgical therapy in treating this life-threatening complication of indwelling catheters.

Kawasaki disease in an 18-month-old boy.

The authors present a case of Kawasaki disease with arrhythmia, aneurysms in the coronary vessels and disease recurrence in week 4. Despite late diagnosis, effective treatment with high doses of immunoglobulin and aspirin was introduced in the second week of the disease as well as during its relapse.

What do we know about Kawasaki disease?

The authors present current theory of the aetiology and pathogenesis of Kawasaki disease with special reference to clinical presentation and cardiac complications. Current therapeutic management has also been discussed.

[Familial hypertrophic cardiomyopathy]. / Rodzinna kardiomiopatia przerostowa.

Investigating the family consisted of 8 members authors confirmed the diagnosis of hypertrophic cardiomyopathy in 5 cases. Secondly clinical features and echocardiographic data were compared. The clinical symptoms were not proportional to the pattern of hypertrophy revealed by echocardiography. The most significant pattern of hypertrophy was registered in youngest patients most likely due to increased expression and penetration of responsible genes. Considering cardiomyopathy as an inherited disease authors find family screening as a key in understanding and management of the disease.

[Diagnostic and therapeutic problems in a newborn with aortic coarctation, adrenal and intracranial hemorrhages and renal failure]. / Problemy diagnostyczne i terapeutyczne u noworodka ze zwezeniem ciesni aorty, wylewami do nadnerczy i komór bocznych mózgu oraz niewydolnoscia nerek.

We report on a 3-day-old newborn with critical coarctation of the aorta, coexisting adrenal and intracranial hemorrhages and acute renal failure requiring dialysis. Severe hypoglycemia, hyperbilirubinemia, mounting anemia and thrombocytopenia, clotting disturbances suggesting DIC, pneumonia, hypertension, increasing circulatory failure and repeated intracranial hemorrhage were observed and were the reason for postponing heart surgery. The child was operated on during the third week of hospitalisation on an emergency basis. The cardiac surgery procedure was performed successfully.

Cerebral infarcts in newborns and infants with cyanotic cardiac anomalies.

Neuropathological examination of six brains of newborns and infants who died in the course of congenital cyanotic cardiac anomalies showed focal brain lesions. The material included five cases from two weeks to two months of age, and one two-year-old infant. In two of them, the periventricular ischemic infarcts were found, in one multifocal encephalomalacia due to multiple vascular occlusions, and in three the necrotic foci corresponded to the supply of large cerebral arteries. The character and topography of severe brain lesions, particularly within the hemispheric white matter, were clearly influenced by the immaturity of the cerebral structures.

[Application of doppler ultrasound in diagnosis of aortic and pulmonary valve stenosis]. / Zastosowanie ultradzwiekowej metody dopplerowskiej w diagnostyce zwezen ujsc tetniczych serca.

120 children (58 boys, 62 girls) aged 2 days-12 years (mean 3.5 +/- 3 years) with pulmonary or aortic valve stenosis underwent the study. Diagnosis was proved by a cardiac catheterization and angiography. There were determinated maximal flow velocities and pressure gradients through stenosed valve using the doppler ultrasound method. Values were compared with those obtained from cardiac catheterization. There were no statistically significant differences between these two methods. Increased blood flow velocities through the aortic or pulmonary valves were stated in the examined group comparing with healthy individuals. Obtained results prove Doppler method usefulness in diagnosis of aortic and pulmonary valve stenosis. In pulmonary valvular and subvalvular stenosis the Doppler method is useful for estimation of outflow tract obstruction while in a extreme case a results can be incompetent.

[Use of Doppler ultrasonography in the diagnosis of aortic coarctation]. / Zastosowanie ultradzwiekowej metody dopplerowskiej w diagnostyce zwezenia ciesni aorty.

Coarctation may be suspected on two-dimensional echocardiography upon notation of an apparent narrowing of the lumen of the aorta, but sometimes the picture is not clear. The purpose of this study was the assessment the detection of coarctation in children with associated defects and if pressure gradient through the obstruction could be accurately estimated from Doppler. Methods. The study included 45 children (38 with coarctation of aorta and 7 with recoarctation) between the ages of 3 days to 14 years. The diagnosis was confirmed by cardiac catheterization and angiocardiography. Doppler echocardiographic studies were performed in all patients before surgery and in 40 patients after surgery. Doppler examination were performed with the use of continuous-wave flow meter Kardiodop KD-84 (Sonopan) with estimation of maximal velocity with histograph Doma HSD-83 (Domar) or with the use of Ultramark 4 Ultrasound System (ATL). The pressure gradient across the stenosis was calculated by using modified Bernoulli equation, and was defined as the subtraction of the square of the maximal velocity x 4 the descending and ascending aorta. Arm and tight cuff sphygmomanometer measurements were performed at the rest. Pressure drop in the descending aorta was measured by sphygmomanometry in 19 children and by catheterization in 10 children. The results were compared with Doppler calculated pressure gradients. Results. The maximal velocity in descending aorta increased on the average to 3.6 +/- 1.2 m/s was recorded in 43 children. With increasing severity velocities were higher, and with severe obstruction a pressure drop across the coarctation may persist throughout diastole and continuous forward flow was seen.(ABSTRACT TRUNCATED AT 250 WORDS)