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Biomedical subjects

F Uhlemann

Publications and source records attributed to F Uhlemann.

23 records · Page 2Linked to original sources

[Interventional high frequency perforation and enlargement of the outflow tract of pulmonary atresia].

In 18 patients with pulmonary atresia interventional perforation of the atresia was attempted using radiofrequency. All patients were referred for palliative surgery. The age ranged from 4 days to 19 years. After angiographic delineation of the relations between the right ventricular outflow tract and main pulmonary artery the tip of a radiofrequency perforation catheter (Cerablate-catheter, 2F, Osypka) was brought into contact with the atresia. The radiofrequency was applied in the unipolar fashion (radiofrequency-generator HAT 200, Osypka). Radiofrequency perforation was successfully performed in 14/18 patients, subsequent balloon dilatation was performed in 12 of these patients. In six patients with muscular atresia a Palmaz-stent was implanted into the right ventricular outflow tract. An inadvertent perforation of the right ventricular outflow tract occurred in three patients, of the pulmonary artery in two patients. However, these were tolerated without sequelae or clinical symptoms. The arterial oxygen saturation increased by 21%; in none of the patients was cardiac failure or pulmonary edema observed. Using the Cerablate-catheter even muscular atresias could be perforated. Implantation of a stent into the newly created right ventricular outflow tract results in a predictable communication between the right ventricle and the pulmonary artery. Although this interventional right ventricular outflow tract creation seems to be a promising new technique, experience is limited and there are no long-term results.

Adolescent↗

Aortic aneurysm rupture in infantile Marfan's syndrome.

A 3-year-old boy with early rupture of an aortic aneurysm due to infantile Marfan's syndrome is presented. In an emergency operation we prepared a composite graft using a 17-mm St. Jude prosthesis with an 18-mm vascular conduit. The postoperative period was complicated by pneumothoraces, transient bilateral phrenic nerve paralysis, cerebral convulsion, and supraventricular tachycardia. Four months postop the composite graft was replaced with an aortic homograft due to severe stenosis. His condition after 12 months is excellent.

Aortic Rupture↗

[Difficulties in diagnosing Marfan's syndrome in childhood and adolescence].

BACKGROUND: The diagnosis of Marfan's syndrome in childhood and adolescence is made by the criteria of the Gent nosology, which evaluates genetic data, family history and a spectrum of clinical criteria. Due to the age dependent manifestations of the clinical symptoms, combined with the extreme heterogeneity of Marfan's syndrome diagnosis in early childhood remains sometimes difficult. PATIENTS: Prospectively, we analyzed the clinical symptoms of all patients where Marfan's syndrome was suspected. We evaluated those patients between January 1997 and April 2002 by an interdisciplinary approach. METHODS: We compared the clinical datas of the patients by using the Gent nosology and the Berlin nosolgy. RESULTS: 34 patients underwent full follow-up. The median age was 10,32 years with a range of 0,01 to 37,31 years, 19 patients were male, 15 patients were female. In eight patients Marfan's syndrome could be rouled out, 9 of 26 patients (34,6 %) fullified the criteria of the Gent nosology, in 17 of 26 patients (65,4 %) Marfan's syndrome remained just suspected, but was not fullified by the criteria of the Gent nosology. Concerning the Berlin nosology 14 of 26 patients (53,8 %) fullified the criteria, 12 of 26 patients (46,2 %) failed. Due to the criteria of the Gent nosology 14 patients (53,8 %) fullified the criteria of skeletal involvement, 21 patients (80,8 %) fullified cardiovascular major manifestation, 6 patients (23,1 %) had an ophthalmic major manifestation, and 9 patients (34,6 %) had an affected first degree relative or were genetically determined. CONCLUSIONS: On the basis of the data of our patients the diagnosis of Marfan's syndrome in childhood and adolescence can be made more sensitive by the criteria of the Berlin nosology compared to the Gent nosology. This seems to be caused by the age dependent manifestations of the symptoms. Until diagnostic algorhythms of Marfan's syndrome in childhood remain suboptimal, continuous clinical follow-up for all cases even those only in the case of suspected Marfan's syndrome are necessary to exclude complicated course and to improve outcome.

Adolescent↗

Mechanical circulatory support for post cardiotomy cardiogenic shock in infants.

Eleven infants weighing 2.3 to 7.8 kg underwent mechanical circulatory support for post cardiotomy cardiogenic shock. Initiated pre-operatively in two patients, extracorporeal membrane oxygenation was used in a total of eight patients aged 6 days to 3 months in association with repair of cyanotic congenital heart disease with increased pulmonary blood flow or with a right sided obstructive lesion. Ventricular assist devices were used in three other patients: a centrifugal left ventricular assist device in Patient 1 (10 months, 5.7 kg) after repair of the anomalous left coronary artery, and a pneumatic biventricular assist device (stroke volume 12 ml) in Patient 2 (6 months, 7.0 kg) for cardiac arrest after closure of ventricular septal defect and in Patient 3 (10 months, 7.8 kg) for post transplant graft failure. Duration of extracorporeal membrane oxygenation duration ranged from 26 to 192 hr (mean, 88 hr). Three patients were weaned from extracorporeal membrane oxygenation and two survived. Two others were separated from extracorporeal membrane oxygenation because of bleeding, but both subsequently died. Patient 1 was weaned from the left ventricular assist device after 192 hr and discharged from the hospital. Support was discontinued after 45 hr in Patient 2 who exhibited irreversible brain damage. Patient 3 was weaned from a biventricular assist device after 174 hr, but suffered recurrent graft failure. Our results show that an appropriate circulatory support system should be selected according to the cardiac anatomy in infants.

Acute Kidney Injury↗