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Biomedical subjects

B Friedli

Publications and source records attributed to B Friedli.

At least 127 records · Page 7Linked to original sources

Complete heart block in a young child presumably due to mycoplasma pneumoniae myocarditis.

The case is described of an 18 months old boy with sudden onset complete heart block, heralded by Stokes-Adams attacks. General signs of viral illness preceded and accompanied the syndrome; this, along with angiographic evidence of poorly contracting left ventricle, led to the diagnosis of non bacterial myocarditis. Serologic tests disclosed a significant rise in antibodies against mycoplasma pneumoniae (1/16 to 1/128). The His-bundle electrogram showed a block above the His-bundle, but fairly widespread damage to the conduction system is suspected. The complete heart block proved to be permanent and a fixed rate pacemaker had to be implanted.

Heart Block↗

Myocardial metabolism in cyanotic congenital heart disease studied by arteriovenous differences of lactate, phosphate, and potassium at rest and during atrial pacing.

To study myocardial metabolism in chronic hypoxia due to cyanotic congenital heart disease, coronary arteriovenous differences of lactate (L), pyruvate (P), inorganic phosphate (Pi) and potassium (K) were measured in 14 cyanotic patients and seven controls, at rest and during atrial pacing. At rest, there was no difference in any parameter between cyanotic and noncyanotic patients. During atrial pacing (150-175/min) for 10 min, a moderate drop in L-extraction occurred in the control patients with some increase in L/P ratio in coronary venous blood. Cyanotic patients fell into two groups: in nine (group I), the arterial oxygen saturation (SaO2) dropped with pacing. Their L-extraction fell sharply, from 28.1 +/- 3.12 to --2.8 5.51 and L production occurred in five. There was a significance increase in coronary venous L/P ratio. Five cyanotic patients (group II) showed no drop in SaO2 with pacing, and L extraction as well as L/P ratio remained stable. Uptake of Pi was noted in all patients at rest, during pacing this disappeared in controls and group I cyanotics but not in group II. No K changes were seen in any patient. Thus, myocardial metabolism is normal at rest in patients with cyanotic CHD; during atrial pacing, a shift toward anaerobic metabolism occurs if SaO2 drops; cyanotic patients whose SaO2 remains stable appear to withstand pacing better than controls.

Adolescent↗

Aortic arch interruption type A with aortopulmonary fenestration in an offspring of a chronic alcoholic mother ("fetal alcohol syndrome").

A case is described of a newborn infant, small for gestational age, with the features of the "fetal alcohol syndrome", who presented a murmur within the first few days of life, and died at age 7 days in heart failure. Autopsy revealed interruption of the aortic arch, type A, an aortopulmonary window and intact interventricular septum. The association of "fetal alcohol syndrome" and cardiac malformation is discussed, with reference to other cases described in the literature.

Adult↗

Hemodynamic studies in children four to ten years after the Mustard operation for transposition of the great arteries.

Fourteen patients have been studied hemodynamically 4-10 years (mean 5.5 years) after the Mustard operation for transposition of the great arteries. Investigation was directed principally at 1) the detection of baffle obstruction by catheterization of the pulmonary veins (PV) and venae cavae and recording of simultaneous right ventricular (RV) and pulmonary capillary wedge pressures (PCW); 2) the detection of intra-atrial baffle leaks by dye curves and selective angiography; 3) the assessment of RV and LV function by calculating peak VCE (dp/dt/28p) from high fidelity recordings in 11 patients. Severe baffle obstruction to the PV return was found in only one patient. The others had no or minimal gradients between RV end-diastolic and PCW pressures (mean 1.3 +/- 0.69 mm Hg). Cardiac output was normal at rest (4.1 +/- .22) and increased to 7.1 +/- .62 L/min/m2 (+73%) but the gradient between the RV end-diastolic and PCW pressures remained insignificant (2.2 +/- 1.13 mm Hg). No evidence of caval obstruction was found in any patient. Baffle leaks were found in five patients with mild bidirectional shunting. All arterial oxygen saturations were above 90%. Mild tricuspid regurgitation was demonstrated in two patients by RV angiography and was absent in 12 others. The contractility index peak VCE averaged 1.87 +/- .122 sec-1 for the RV and was significantly lower in the LV (1.53 +/- 1.35 SEC-1, P less than 0.01). Only one patient presented significantly decreased RV contractility with a peak VCE of 1.07 sec-1 and poor contraction on the RV angiogram. These data indicate that the long-term prognosis after the Mustard operation should be good in most patients and that the right ventricle is capable of functioning at the level of contractility of a normal left ventricle.

Angiocardiography↗

Sinus node function and conduction system after complete repair of tetralogy of Fallot.

Twenty-three children underwent electrophysiological studies during routine postoperative catheterization two months to five years after complete correction of tetralogy of Fallot. The aim of the study was to investigate the whole conduction system, including sinus node function, suing His bundle recordings and atrial pacing. H-V intervals were normal at rest and with pacing in twenty-two patients, including four patients with evidence of bifascicular block on the surface ECG. One patient with cardiomegaly and evidence of diffuse myocardial damage had a prolonged H-V interval but did not develop a block at this level during pacing A-H interval was slightly prolonged in four patients and normal in all others. The threshold of pacing-induced atrioventricular block ranged from 75 to 240/min and was somewhat age dependent (r = -0.55). Two patients fell below the 95% confidence limit of this regression and are considered abnormal. One returned to normal after intravenous injectionof atropine. Corrected sinus node recovery time ranged from 60 to 2000 msec. Three patients had values above 500 msec which are considered abnormal. These patients had other minor signs of sinus node dysfunction, i.e., episodes of sino-atrial block at rest or intermittent sinus bradycardia. Thus, while the His Purkinje system performed satisfactorily in all patients, sinus node dysfunctional and A-V node dysfunction were demonstrated in a few patients after correction of tetralogy of Fallot.

Adolescent↗

The effect of increased pulmonary blood flow on the pulmonary vascular bed in pigs.

Increased pulmonary blood flow was produced in 1-month-old piglets by means of left pneumonectomy, arteriovenous fistulas in the neck, and a combination of both. Physiologic and histologic studies of the pulmonary vascular bed were done 1-9 months after operation. A progressive, moderate increase in pulmonary artery (PA) pressure was observed, especially between 1 and 6 months after surgery. This was flow related, i.e., the group with the highest flow (pneumonectomy plus fistula) was found to have the most prominent increase in pressure. Mean Pa pressure at 6 months was 28.7 +/- 0.07 mm Hg in this group, vs 24.4 +/- 0.48 mm Hg in the group with pneumonectomy alone and 17.2 +/- 0.48 mm Hg in controls (P less than 0.01). The pressure response to hypoxia in pigs with high pulmonary blood flow was not different from that found in control animals. Histologic studies revealed that small arteries and arterioles of pigs with high pulmonary blood flow had a decreased relative wall thickness because of dilation up to 6 months follow-up. This was flow related, the group with the highest flow having the lowest wall thickness to vessel diameter ratio; relative wall thickness (in percentage of the vessel diameter ) at 6 months was 6.1 +/- 0.44% in pigs with with pneumonectomy plus fistula, vs 9.6 +/- 0.40% in the group with pneumonectomy alone and 11.2 +/- 0.61% in controls (P less than 0.01). In the group with the highest flow, thick walled arterioles appeared at 9 months follow-up, scattered among dilated ones; between 6 and 9 months after operation, ranging from 6.1 +/- 0.44% to 11.3 +/- 0.73% (P less than 0.01). In five animals with high flow, the right PA (main branch) showed patchy intimal thickening, small cystic spaces filled with mucopolysaccharides in the media, and muscular hypertrophy.

Animals↗

Systemic-to-pulmonary artery fistula following actinomycosis.

We report a case of pleuropulmonary actinomycosis in a child followed by a most unusual complication: the appearance of a systemic-to-pulmonary artery fistula in the area where the abscess was drained. Fifteen months after successful treatment with penicillin and surgical drainage, a continuous murmur was heard over the scar. Aortic angiography showed multiple connections between the intercostal arteries and the left pulmonary artery through an angiomatous lesion in the left lower lobe. This resulted in a considerable left-to-right shunt. Possible pathogenic mechanisms are discussed.

Actinomycosis↗