Congenital coronary artery fistula in infancy and childhood: diagnostic and therapeutic aspects.

Clinical symptoms and age at manifestation of a congenital coronary artery fistula may vary considerably. They depend on the underlying anatomy and also on the size of the fistulous connection to the left or right side of the heart. Using colour Doppler echocardiography for direct visualization of the entire course of the fistulous vessel, including the site of termination, succeeds only in a small number of cases. Furthermore, regular coronary vessels branching off proximally and distally of the coronary artery fistula usually are not recognizable by this method. Only selective angiography provides this information and is unchallenged the most important and indispensable diagnostic technique, especially with regard to surgical treatment. This publication presents physical, echocardiographic, and angiographic data of 15 patients, who were admitted to the German Heart Center Munich between 1970 and 1993. By an invasive diagnostic approach the following arteriovenous fistulous connections were found: from right coronary artery to right atrium (3 patients) or to right ventricle (3 patients), from left coronary artery to right atrium or coronary sinus (3 patients), from left coronary artery to right ventricle (4 patients) and from right and left coronary artery to right ventricle (2 patients). In 5 patients a "proximal" form of coronary artery fistula ("side-to-side pattern") was found, in 8 patients a "distal" form ("end-artery type"), and in 2 patients a combination of both forms. In 14 patients surgical closure was performed (6 symptomatic infants, mean age at surgery = 95 days, and 8 asymptomatic children, mean age at surgery = 7.1 years): 13 patients survived surgery. On an average of 5 years after surgery all of these 13 patients are in excellent condition (NYHA functional class I). The experiences in surgical treatment verify the importance of an exact angiographic visualization of the anatomy of a coronary artery fistula and the regular coronary vessels branching off proximally and distally of the fistula. Closure of coronary artery fistulas at the time of diagnosis is recommended also in asymptomatic patients, since perioperative morbidity and mortality increases in older patients.

Randomized controlled trial of Ringer solution versus serum for partial exchange transfusion in neonatal polycythaemia.

UNLABELLED: We tested whether crystalliod solutions could be used instead of colloid solutions for partial exchange transfusions (PET) in polycythaemic neonates because crystalloid solutions are cheap, carry no risk of anaphylactic reactions and can be sterilized. We randomly assigned 20 term neonates with venous haematocrit (Hct) > 0.65 l/l to PET with either a serum preparation (BISEKO) or Ringer solution. Plasma volume (PV) was measured with Evans blue dilution. Blood volume (BV) and red cell mass were calculated from PV and venous Hct. Before PET both serum and Ringer groups had the same Hct (0.69 (0.66-0.76) vs 0.69 (0.66-0.71) l/l; median (range)) and BV (108 (81-116) versus 96 (68-121) ml/kg. During PET an equivalent amount of blood was withdrawn stepwise (19 (14-26) versus 17 (13-25) ml/kg and replaced by either serum or Ringer solution. More of the Ringer solution (median 77%) than of the serum (median 36%) given left the intravascular space within 4 h after PET (P = 0.016); but there was no significant difference in Hct after Ringer-PET compared to serum-PET (median 0.58 vs 0.56 l/l). No infant required repeat PET. Ringer-PET reduced BV from high to normal values (from median 96 to 83 ml/kg; P = 0.005), whereas after serum-PET BV remained high (from median 108 to 98 ml/kg; not significant). CONCLUSION: PET with Ringer solution resulted in a haemodilution comparable to PET with serum and a correction of hypervolaemia.

Airway pressure measurements during high-frequency positive pressure ventilation in extremely low birth weight neonates.

OBJECTIVES: Evaluation of a modified, computer-controlled, shutter method to determine the complete intrapulmonary pressure course and to ascertain the expiratory time constant for the respiratory system during high-frequency positive pressure ventilation. DESIGN: Prospective clinical study. SETTING: Neonatal intensive care unit in a university hospital. PATIENTS: Sixteen premature newborns (mean gestational age 26 +/- 2 [SD] wks, birth weight 741 +/- 138 g) were studied at various times during their clinical course. MEASUREMENTS AND RESULTS: Installation of the shutter and air flow interruption did not result in any impairment of clinical and respiratory conditions. Time constants were between 58 and 190 msecs. In six patients, an inadvertent positive end-expiratory pressure (1 to 4.5 cm H2O) was found; in these patients only, expiratory time set at the respirator was < 4 time constants. In 13 measurements of nine patients, measured intrapulmonary peak inspiratory pressure was considerably lower (1 to 5 cm H2O) than that value set at the respirator. CONCLUSIONS: The computer-controlled shutter method is noninvasive and applicable without impairment, even in preterm neonates with birth weights of < 1000 g. This method provides important information to optimize respiratory therapy, particularly knowledge of the individual time constant. To avoid inadvertent positive end-expiratory pressure and gas trapping, expiratory time should be > 4 time constants.

[Postnatal weight loss in ventilated premature infants below 1500 g: significance of renal and extra-renal fluid loss]. / Postnatale Gewichtsabnahme beatmeter Frühgeborener unter 1500 g: Bedeutung des renalen und extrarenalen Flüssigkeitsverlusts.

In 9 ventilated preterm infants (birth weight 810-1310 g, gestational age 26-30 weeks) we measured total body water (D2O dilution), extracellular volume (sucrose dilution) and renal function parameters at birth and when postnatal weight loss exceeded 5% of birth weight together with day to day changes of weight, fluid balance, and serum electrolytes. A significant postnatal weight loss (-89 +/- 47 g) occurred together with a reduction of total body water (-102 +/- 95 ml) and extracellular volume (-98 +/- 59 ml) in the first three days of life. The intracellular volume did not change. At the same time fluid balance was negative due to a high extrarenal water loss on day 1 and an increasing diuresis on the following days. The serum sodium concentration remained unchanged during postnatal water loss, because the negative fluid balance was accompanied by a negative sodium balance.

Body composition, nutrition, and fluid balance during the first two weeks of life in preterm neonates weighing less than 1500 grams.

To determine whether body weight during the first 2 weeks of life in preterm infants weighing less than 1500 gm reflects nutritional status or fluid balance, we studied total body water (TBW) (deuterium oxide dilution), extracellular volume (sucrose dilution), and plasma volume (Evans blue dilution), together with intake-output studies of nitrogen, fluid, and sodium on day 1 (median age 0.3 day), at a weight loss of 7.8% of birth weight (median age 3.4 days), and after birth weight was regained (median age 8.9 days) in eight clinically stable preterm infants (birth weight 810 to 1310 gm, gestational age 26 to 30 weeks) receiving ventilatory support. During the initial weight loss we found no evidence of catabolism. Body solids (weight--TBW) remained unchanged, there was nitrogen retention, and energy intake was sufficient to meet energy expenditure by day 2. However, we found evidence of fluid loss: TBW (mean +/- SD, -95 +/- 99 ml), extracellular volume (-98 +/- 63 ml), and interstitial volume (-102 +/- 75 ml) decreased significantly, indicating negative fluid and sodium balances. Blood volume and plasma volume remained unchanged. With the regaining of birth weight there was no increase in body solids despite a high degree of nitrogen retention, but there was a positive fluid balance although no significant increase in any body fluid compartment was found. We conclude that the observed postnatal weight changes reflect changes in interstitial volume.

[Psychosocial dwarfism]. / Psychosozialer Minderwuchs.

A 4 year old girl with psychosocial dwarfism showed hepatomegaly, abdominal distension and behavioral disturbances. Laboratory tests revealed a reversible elevation of serum liver enzymes and a reversible partial growth hormone deficiency. The child's family was characterised by severe social problems and all signs of a depriving and child abusing environment. All pathologic findings were reversed by removing the child from her depriving family background. This case demonstrates that psychosocial dwarfism represents an aspect of child abuse (battered child syndrome).