Continuous extracorporeal fluid removal in children with low cardiac output after cardiac operations.

Eleven hypervolemic and oliguric children with low cardiac output after cardiac operations were treated by slow continuous ultrafiltration or continuous arteriovenous hemofiltration. A mean negative fluid balance of 1.63 +/- 0.37 ml/kg/hr (standard error of the mean [SEM]) significantly improved the hemodynamic status within 59 +/- 6.1 hours (SEM). Although the central venous pressure decreased significantly from 15.2 +/- 0.84 to 8.8 +/- 0.92 mm Hg (p less than 0.0001), the mean arterial pressure increased significantly from 41.5 +/- 2.54 to 53.5 +/- 2.21 mm Hg (p less than 0.001). In addition, pH increased significantly from 7.31 +/- 0.01 (SEM) to 7.43 +/- 0.001 (SEM) (p less than 0.001) and oxygenation index (arterial oxygen tension/inspired oxygen fraction) from 119 +/- 15.2 (SEM) to 214 +/- 27.0 (SEM) (p less than 0.001). Hemodynamic improvement during slow continuous extracorporeal fluid removal allowed a significant decrease of the catecholamine infusion rate. After normovolemia had been achieved, continuous arteriovenous hemofiltration had to be continued in four children because of persistent anuria. Eight patients could be weaned from artificial ventilation and vasopressor support. Two patients died without recovery of renal function and one with restored renal function. Slow continuous ultrafiltration and continuous arteriovenous hemofiltration improve the cardiovascular function in children with low cardiac output by optimizing the preload conditions of the failing heart. In addition, they improve acid-base balance and pulmonary gas exchange.

Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis.

OBJECTIVE: The aim of this study was to evaluate the effects of inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. METHODS: Inhaled nitric oxide (mean 4.1 +/- 0.7 ppm, 1.5 to 10 ppm) was administered in 13 patients (mean age 5.6 +/- 1.6 years, 1.5 to 17 years) with critical pulmonary perfusion (central venous pressure > 20 mm Hg or transpulmonary pressure gradient > 10 mm Hg) in the early postoperative period after total cavopulmonary connection (n = 9) or after bidirectional Glenn anastomosis (n = 4). RESULTS: In patients after total cavopulmonary connection inhaled nitric oxide therapy decreased central venous pressure by 15.3% +/- 1.4% (p = 0.0001) and transpulmonary pressure gradient by 42% +/- 8% (p = 0.0008) and increased mean systemic arterial and left atrial pressures by 12% +/- 3.6% (p = 0.011) and 28% +/- 8% (p = 0.007), respectively. Arterial and venous oxygen saturations improved by 8.2% +/- 1% (p = 0.005) and 14% +/- 4.3% (p = 0.03), respectively. In patients after bidirectional Glenn anastomosis inhaled nitric oxide therapy resulted in a decrease of central venous pressure by 22% +/- 1% and of the transpulmonary pressure gradient by 55% +/- 6% and improved arterial and venous oxygen saturations by 37% +/- 29% and 11% +/- 3%, respectively. Mean systemic arterial and left atrial pressures remained nearly unchanged. No toxic side effect was observed in any patient. CONCLUSION: Inhaled nitric oxide may play an important role in the management of transient critical pulmonary perfusion caused by reactive elevated pulmonary vascular resistance in the early postoperative period after Fontan-type operations and bidirectional Glenn anastomosis.

Transcatheter coil occlusion of residual interatrial communications after Fontan procedure.

OBJECTIVE: To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. DESIGN: Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. SETTING: Tertiary paediatric cardiac referral centre. PATIENTS: Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. INTERVENTIONS: Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. RESULTS: A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range 86-89%) to 92 (1.3)% (range, 89-93%; p < 0.001) and the mean (SD) right atrial pressure rose from 9.7 (2) mm Hg (range, 6-11) to 10.6 (2.4) mm Hg (range, 6-13; p < 0.05) after coil implantation. In five patients, complete obliteration of the interatrial shunt was shown by angiography after coil implantation. At a mean (SD) follow up of 10 (4) months (range, 3-15) a residual interatrial shunt was detected by Doppler colour echocardiography in only one patient, and oxygen saturations ranged from 90% to 95% (mean, 92%). There were no late coil embolisations, thromboembolic events, or haemolysis in any patient. CONCLUSIONS: Detachable coils can be used successfully to occlude residual interatrial communications after the Fontan procedure. In selected cases, in whom intended transcatheter umbrella occlusion of residual interatrial leaks is not possible, the use of detachable coils might offer a safe alternative method to eliminate interatrial right to left shunting after the Fontan procedure.

Double outlet left ventricle with intact ventricular septum.

Two cases of double outlet left ventricle with intact ventricular septum are reported. Both patients presented with neonatal cyanosis and signs of right ventricular outflow tract obstruction. Both have hypoplastic right ventricles with suprasystemic pressures, pulmonary to mitral valve continuity and the aorta in left-sided position. These seem to be the second and third cases of the malformation to be described thus far.

Mitral atresia with premature closure of the oval foramen, right-sided levoatriocardinal vein and thrombus formation in the left atrium.

We describe a 4-week-old baby with mitral atresia, hypoplastic left ventricle, ventricular septal defect, preductal coarctation and premature closure of the oval foramen whose only outlet from the left atrium was a stenosed right-sided levoatriocardinal vein and who in addition developed left atrial thrombi. Cross-sectional echocardiography was extremely helpful in establishing the diagnosis.

Balloon atrial septostomy under two-dimensional echocardiographic control.

We report our experience with balloon atrial septostomy under two-dimensional echocardiographic guidance only in 44 newborn patients (39 patients with transposition of the great arteries) who needed creation of an interatrial communication. Using standard echocardiographic projections septostomy with creation of large atrial septal defects was easy in 41 newborns. In 3 patients insertion of a large Eustachian valve at the interatrial septum prevented adequate pull-throughs with larger balloon sizes. No serious complications due to the procedure were noted. Balloon atrial septostomy under two-dimensional echocardiographic control is a quick, effective and safe method which can be performed on the intensive care unit.

Successful palliation of the "absent" pulmonary valve syndrome by banding of the pulmonary trunk.

The "absent" pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary trunk, stenosis of the ventriculo-arterial junction with or without malalignment of the outlet septum, and ventricular septal defect. When the outlet septum is malaligned, the morphology resembles that of tetralogy of Fallot. We report our experience with 4 infants with this syndrome. All were in severe respiratory distress and cardiac failure when first seen. Cardiac catheterization was performed at 0.5-4.5 months of age in 3 of them. In the other, the clinical and echocardiographic features were considered sufficient to establish the diagnosis. Banding of the pulmonary trunk was carried out at the age of 2.5-5 months. The distal pulmonary arterial pressure in 3 cases dropped to 12-19 mm Hg. These patients could be extubated within one week postoperatively. Their course 1-3 years later is excellent, with rare episodes of mild respiratory problems only and markedly diminished pulmonary insufficiency. One child, weighing 3250 g at surgery, whose pulmonary arterial pressure did not drop below 29 mm Hg, could not be weaned off the respirator. Corrective surgery was undertaken 17 days later, but the patient died of respiratory complications. Based on clinical and Doppler sonographic findings, on control catheterization data and on haemodynamic findings in 3 surviving infants and two further patients with an uneventful course who, as yet, have not undergone surgery, we conclude that the beneficial effect of banding is the combined result of reduced pulmonary arterial pressure and decreased pulmonic regurgitation.

False passage in the interventricular septum. Echocardiography in diagnosis and management of a rare complication after closed transventricular valvotomy for critical aortic stenosis.

A case of a false passage causing an aneurysm of the interventricular septum is reported. Two-dimensional echocardiography facilitates the correct preoperative diagnosis of critical aortic stenosis and is helpful in the postoperative management to assess the outcome and detect complications. Surgical intervention for a false passage was prevented by serial echocardiographic follow-up studies showing resolution of the aneurysm.

CT diagnosis of thrombosis of dural sinuses in childhood.

This paper describes the CT findings in five cases of cerebral and cerebellar sinovenous occlusion. Characteristic CT findings are the visualisation of the thrombosed sinuses and veins, known as the "filled triangle" and the "cord sign" on the control scan, and the "empty triangle" with tentorial and gyral enhancement after contrast enhancement. The high incidence of thrombosed internal cerebral veins and superior cerebellar veins with sinus thrombosis in children is remarkable. CT with both control and contrast enhanced scans will probably provide the correct diagnosis in the majority of cases.

[Angiographic-clinical correlation in Fallot's-tetralogy during childhood (author's transl)]. / Angiokardiographisch-klinische Korrelationen bei Fallotscher Tetralogie im Kindesalter.

Thirty-eight patients with Fallot's tetralogy were investigated in order to correlate their clinical and angiographic findings. In the light of embryological knowledge about the morphogenesis of Fallot's tetralogy there are good explanations for the various modes of clinical presentation. Patients with extreme cyanosis in the newborn period show diffuse hypoplasia of their right ventricular outflow tract and pulmonary arteries because of extreme malseptation of the conus with anteposition of the infundibular septum. Patients who present with cyanosis and blue spells in infancy show infundibular narrowing and dextro- and anteposition of the ascending aorta. Conal malseptation together with malrotation of the conus seem to be the responsible factors in this patient group. Malrotation of the conus without malseptation is probably the mechanism in patients who present initially without cyanosis and signs of left to right shunting. Anteposition and overriding of the aorta are the initial angiographic signs while right ventricular hypertrophy producing infundibular narrowing and subsequent cyanosis appear later.

[Percutaneous balloon dilatation of pulmonary valve stenosis]. / Perkutane Ballondilatation valvulärer Pulmonalstenosen.

Balloon valvuloplasty was carried out on five children with isolated valvular pulmonary stenosis. One child had a residual stenosis following a Brock's transventricular valvotomy. The systolic gradient between the right ventricle and the pulmonary artery fell from 60.4 +/- 18.7 mmHg to 21.8 +/- 12.0 mmHg. Right ventricular pressure fell from 76.2 +/- 20.7 mmHg to 43.0 +/- 16.4 mmHg. Percutaneous balloon valvuloplasty has proved to be an effective method for the treatment of isolated valvular pulmonary stenosis.

[Scimitar syndrome with horseshoe lung (author's transl)]. / Scimitarsyndrom mit Hufeisenlunge.

A combination of two rare malformations of the lung was observed in a four-year-old asymptomatic boy. He had typical scimitar syndrome (dextrocardia, hypoplastic right lung and right-sided anomalous pulmonary venous drainage into the v. cava inferior) together with horseshoe lung. Diagnosis was established by angiocardiography and computerized tomography. In the absence of recurrent pulmonary infections operative intervention is not necessary with normal pulmonary arterial pressure and resistance. To the best of our knowledge this case with both malformations seems to be the fourth which is reported in the literature.

[Deformities of the spine and ribs in embryologically related malformations of the heart with cyanosis]. / Wirbelsäulen-und Rippenmissbildungen bei embryologisch verwandten zyanotischen Herzfehlern

Three cases of severe costovertebral deformities together with embryologically related cardiac malformations (Fallots Tetralogy and Truncus arteriosus communis) are presented. Two patients died because of their thoracic deformities and limited respiratory function. Relationships to similar bizarre veretebral anomalies are discussed. The cause is probably a teratogenic agent, acting between the fifth to eight embryologic week.

[The syndrome of polysplenia (author's transl)]. / Das Polyspleniesyndrom.

A strong tendency to symmetrical development of the organ systems manifests itself in the syndrome of Polysplenia. These symmetrical organs show the anatomy of leftsided structures. For this reason the syndrome is also referred to as the "syndrome of bilateral leftsidedness". It encompasses complex abnormalities of situs, typical cardiac and vascular deformities as well as extracardiac malformations. Polysplenia is the leading symptom. Diagnosis is rarely made intra vitam. Certain typical deformities can give hints to this diagnosis, which is finally proved by szintigraphic and arteriographic visualization of multiple spleens. A typical case is reported and subsequently discussed according to the picture of this syndrome in the literature.

Prospective evaluation of clinical scoring systems in infants and children with cardiopulmonary insufficiency after cardiac surgery.

OBJECTIVE: To document severity of illness and to evaluate the predictive value of clinical scoring systems in infants and children with cardiopulmonary insufficiency after cardiac surgery. DESIGN: Prospective study with follow up to hospital discharge. SETTING: A multidisciplinary pediatric ICU in a University Hospital. PATIENTS: Between 1/1989 and 4/1992 441 infants and children with congenital heart disease underwent open heart surgery. 128 of these patients developed postoperative cardiopulmonary insufficiency and were entered into this study. METHODS: Data relevant to the Acute Physiologic Score for Children (APSC), Pediatric Risk of Mortality (PRISM), Therapeutic Intervention Scoring System (TISS) and Organ System Failure (OSF) score were collected in all patients during the first 4 days of postoperative intensive care. RESULTS: The mean age of the patients was 1.5 +/- 0.2 years. The mean duration of mechanical ventilation and ICU care was 6.2 +/- 0.6 and 8.1 +/- 0.7 days, respectively. On the first postoperative day the mean APSC and PRISM scores of survivors and nonsurvivors were 13.9 +/- 1.3 vs 24.5 +/- 1.3 (p < 0.001) and 6.1 +/- 0.5 vs 19.6 +/- 1.9 (p < 0.001), respectively. The mean TISS and OSF scores of survivors and nonsurvivors were 46 +/- 0.8 vs 57.8 +/- 1.4 (p < 0.001), and 2.2 +/- 0.2 vs 3.4 +/- 0.2 (p < 0.001), respectively. The overall hospital mortality rate was 9.9%, the hospital mortality rate of patients with postoperative cardiopulmonary insufficiency 34%. Patients with an APSC score < 10 and a PRISM score < 5 had a survival rate of 100%, whereas patients with an APSC score > 30 and a PRISM score > 25 had a mortality rate of 100%. The area under the receiver operating characteristic (ROC) curve for APSC, PRISM and TISS was 0.847, 0.826 and 0.793, respectively. CONCLUSION: APSC, PRISM and TISS describe accurately severity of illness in infants and children with cardiopulmonary insufficiency after cardiac surgery and all scores identify those patients at increased risk for mortality.

Inhaled nitric oxide in infants and children after open heart surgery.

OBJECTIVE: To assess the effects of inhaled nitric oxide (NO) on oxygenation and pulmonary circulation in infants and children with critical pulmonary perfusion and/or hypoxemia after open heart surgery. STUDY: A prospective case series report. SETTING: A multidisciplinary pediatric intensive care unit in a University hospital PATIENTS: From June 1993 to March 1996 37 pediatric patients after open heart surgery were treated with inhaled NO. Their mean age was 2.9+/-0.6 years, their mean body weight 12.6+/-1.8 kg. METHODS: Inhaled NO was applied using a microprocessor controlled delivery system which continuously measured NO and NO2 by the chemilumniscence method. Monitoring included ECG, continuous pulse oximetry (SaO2), arterial (AP), central venous (CVP) and left atrial (LAP) pressures and in 8 patients a pulmonary artery (PAP) pressure. Inhaled NO was started at an SaO2 <90% with a fraction of inspired oxygen concentration (FiO2) >0.7, at a mean pulmonary artery pressure (MPAP) >50% of the mean arterial pressure (MAP), and in patients after Fontan-procedure at a CVP-LAP pressure gradient >10 mmHg. RESULTS: The mean dose of inhaled NO was 3.7+/-0.3 ppm and the mean duration was 112+/-14.7 hours. For the whole group SaO2 increased from 79.6+/-2.3 to 90.1+/-1.5% (p<0.01) within 20 minutes of NO-inhalation. Inhaled NO significantly decreased the MPAP from 47.8+/-4 to 27.5+/-2.3 mmHg (p<0.01) in 8 patients with postoperative pulmonary hypertension and significantly decreased the transpulmonary pressure (CVP-LAP) from 14.3+/-0.8 to 7.3+/-0.9 mmHg (p<0.01) in 16 patients after Glenn- or Fontan-procedure. CONCLUSIONS: Inhaled NO is very effective to decrease pulmonary artery pressure, to improve oxygenation, and to improve Fontan-circulation in infants and children after open heart surgery.

[Possibilities of 2-dimensional echocardiography in childhood. II. Possibilities for use]. / Möglichkeiten zweidimensionaler Echokardiographie im Kindesalter. II. Anwendungsmöglichkeiten.

Two-dimensional echocardiography has been shown to be equal, or even superior, to cardiac catheterization in the diagnosis of congenital heart disease. On the basis of clinical and non-invasive investigations alone many cardiac malformations can be diagnosed correctly and an operation carried out without previous catheterization. This paper reports our findings with cross-sectional echocardiography in common and rare cardiac malformations using standardized imaging planes and typical transducer locations.

[Persistent pulmonary hypertension in newborn infants (author's transl)]. / Persistierende pulmonale Hypertonie des Neugeborenen.

Three cases of persistent pulmonary hypertension in newborn infants are reported. One patient developed this syndrome following a normal pregnancy and uneventful delivery, whilst the others had a history of fetal or perinatal distress. Cardiac catheterization showed pulmonary hypertension in two cases in the absence of cardiac or pulmonary disease or metabolic disorders; one of these babies died unexpectedly following initial improvement. The second infant died from massive cardiac failure before cardiac catheterization could be carried out. The surviving infant was reinvestigated at the age of 7 months and showed normal pulmonary pressure and no evidence of cardiac disease. Aetiology, haemodynamics and clinical picture of this syndrome are discussed in relation to the varying clinical features manifested by our patients and the course taken.

[Possibilities of 2-dimensional echocardiography in childhood. I. Study technic, section planes, normal findings]. / Möglichkeiten zweidimensionaler Echokardiographie im Kindesalter. I. Untersuchungstechnik, Schnittebenen, Normalbefunde.

Two-dimensional echocardiography is a superb method for non-invasive investigation of cardiac anatomy and cardiac function in infancy and childhood. It supplements and often replaces invasive investigations. This paper presents the standardized imaging planes and transducer locations and shows the normal cross-sectional cardiac anatomy.