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Biomedical subjects

I Luhmer

Publications and source records attributed to I Luhmer.

At least 19 recordsLinked to original sources
PubMed2004

Excision of the coronary orifices in arterial switch operation: "O" like obstructive and "U" like unobstructive?

BACKGROUND: Stenoses of the neo-pulmonary artery (NPA) may complicate follow-up of the arterial switch operation (ASO). It is unknown whether the type of patch covering the coronary excision defects ("O"- or "U"-shaped) might influence this complication. METHODS: Echocardiographically and invasively measured NPA pressure-gradients were evaluated retrospectively in 95 children after ASO. Median follow-up was 5.8 years. Defects had been covered with pericardial patches: O/O and U/U (left/right) 34 x each, and 27 x in mixed combinations. The frequency of NPA stenoses requiring re-interventions was registered. RESULTS: Median of the peak instantaneous echocardiographic pressure gradient was 23 mmHg (interquartile range, IQR: 16 - 49, n = 34) in O/O, and 19 mmHg (IQR: 13 - 23, n = 34) in U/U; p < 0.034, t-test. Invasively measured gradients were 49 mmHg (IQR: 17 - 65, n = 12) in O/O, and 12 mmHg (IQR: 7-21, n = 28) in U/U; p < 0.001. One child per O/O- and U/U-group underwent balloon angioplasty of the neo-pulmonary root. Five children of the O/O-group had to undergo repeat surgery, whereas only one child in the U/U-group required repeat surgery ( p < 0.34). CONCLUSIONS: The preferential type of covering the NPA coronary excision sites in ASO should be U-shaped.

Anastomosis, Surgical↗
PubMed1999

A search for chromosome 22q11.2 deletions in a series of 176 consecutively catheterized patients with congenital heart disease: no evidence for deletions in non-syndromic patients.

Microdeletions in chromosome 22q11.2 are associated with DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and several other syndromes, collectively referred to as DG/VCF. Non-dysmorphic patients with cardiac defects have also been attributed to deletions in this chromosomal region. In this study 157 consecutively catheterized patients with isolated, non-syndromic cardiac defects, and 25 patients with cardiac defects and additional stigmata (10 of whom were clinically diagnosed as DG/VCF cases prior to chromosome analysis) were analysed by fluorescence in situ hybridization with the DGS-specific probe D0832. Chromosome 22q11.2 deletions were observed only in the ten patients with the clinical diagnosis of DG/VCF. Conclusion In a large unselected cohort of patients with congenital heart disease no association between isolated or non-syndromic heart defects and the 22q11.2 microdeletion was observed. One can conclude that testing for the 22q11.2 microdeletion is clearly indicated in cases when even mild extracardiac abnormalities are present, particularly in very young infants.

Adolescent↗
PubMed1999

Instantaneous pressure-flow velocity relations of systemic venous return in patients with univentricular circulation.

OBJECTIVE: To assess the pressure and flow velocity relations and respiratory variability of the systemic venous and hepatic venous return in patients with univentricular circulation. PATIENTS: 15 selected patients who had undergone cavopulmonary anastomosis (10) or atriopulmonary anastomosis (5). Mean age at operation was 55.1 months (range 9 to 145). Studies were done at 75.5 (32.6) months (mean (SD)) after the operation. SETTING: Tertiary referral centre. METHODS: Patients were studied using simultaneous recordings of ECG, pressure trace, respirometer trace, and pulsed Doppler echocardiography. Mean systemic venous pressure and pulmonary vascular resistance did not differ significantly between the two patient groups. RESULTS: After total cavopulmonary anastomosis, systemic venous pressure tracings showed a flattened pressure curve without any dependence on cardiac or respiratory cycle. After atriopulmonary anastomosis, right atrial pressure tracings showed a significantly higher "a" wave corresponding to atrial contraction, without any respiratory variability. Pulsed Doppler examination of the superior and inferior caval vein and hepatic vein after total cavopulmonary anastomosis did not show a reverse flow after atrial contraction. The inspiratory to expiratory velocity ratio of antegrade flow revealed a significant dependence of flow on changes in intrathoracic pressure in the intra-atrial tunnel, caval veins, and hepatic vein. During expiration, decrease or cessation of antegrade hepatic venous flow was documented. After an atriopulmonary anastomosis, there was a biphasic antegrade venous flow pattern without significant respiratory variation. CONCLUSIONS: After total cavopulmonary anastomosis, there was marked respiratory dependence of systemic and hepatic venous return, whereas after an atriopulmonary anastomosis venous flow pattern varied according to cardiac cycle and pressure trace. The effects of total cavopulmonary anastomosis on venous return might counteract its other haemodynamic advantages.

Blood Flow Velocity↗
PubMed1998

[High frequency catheter ablation in young patients with permanent junctional reentry tachycardia and ectopic atrial tachycardia].

Ten young patients with a median age of 10.5 (range: 6 to 31) years suffering from the permanent form of junctional reciprocating tachycardia (PJRT; n = 7) and ectopic atrial tachycardia (AET; n = 3) were treated by transcatheter radiofrequency current application. Indications for interventional therapy were failure of medical therapy after a median of 3 antiarrhythmic drugs in all patients, syncope in 1, and impaired left ventricular function in 4 patients (PJRT n = 3, AET n = 1). The intervention was primarily successful in all patients. Median fluoroscopy time during the ablation procedure was 17 (10 to 70) minutes. A median of 3 (1 to 14) radiofrequency (500 kHz) pulses was delivered at a target temperature of 70 degrees C or with 30 W for 30 s. One patient had a recurrence of PJRT and was successfully treated by a repeat ablation. After a median follow-up of 14 (2 to 61) months, all patients are free of drug treatment and in normal sinus rhythm. Left ventricular function has normalized in patients with follow-up > 6 months. Radiofrequency catheter ablation was a safe and effective therapy in young patients with drug-resistant forms of chronic-permanent supraventricular tachycardia.

Adolescent↗
PubMed1998

Pulsed Doppler echocardiographic assessment of patterns of venous flow after the modified Fontan operation: potential clinical implications.

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs. Significantly lower maximum velocities of flow at inspiration (0.31+/-0.12 m/sec vs 0.45+/-0.14 m/sec) and expiration (0.23+/-0.09 m/sec vs 0.32+/-0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastomosis (p = 0.001 and p < 0.001, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5+/-0.21 and 0.57+/-0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group. The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastomosis may influence hepatic function in the postoperative period.

Adolescent↗
PubMed1997

[Total cavopulmonary anastomosis for definitive palliation of complex univentricular cardiovascular systems--potential preoperative risk factors].

The modified Fontan operation for definitive palliation has been performed in an increasing number of patients with various forms of a functionally univentricular heart. To eliminate the influence of different surgical techniques only patients after total cavopulmonary anastomosis (TCPA) were included in this retrospective analysis of preoperative risk factors. The age at operation in 72 patients varied from 7 to 219 months (median 54 months); 29 patients had been younger than 4 years. Forty-nine patients presented with a complex form of a functionally univentricular heart; associated systemic- or pulmonary venous anomalies were found in 22 patients, AV valve abnormalities in 42 patients with AV valve incompetence in 21 patients. Preoperative hemodynamic data revealed an elevated mean pulmonary artery pressure (> 15 mm Hg), increased pulmonary arteriolar resistance (> 3 U.m2) or end-diastolic ventricular pressure (> 12 mm Hg) in 23 patients. The overall mortality was 9.7% (7/72 patients). Variables with significant influence on postoperative mortality were associated systemic- and pulmonary venous anomalies. AV-valve incompetence and prolonged cardio-pulmonary bypass time. Age at operation, preoperative pulmonary arteriolar resistance, systemic- and pulmonary blood flow did not influence postoperative mortality. Even in patients with a complex form of a univentricular heart with associated anomalies and borderline preoperative hemodynamics TCPA can be performed with an acceptable risk.

Adolescent↗
PubMed1997

[Evaluation of congenital vena cava anomalies and acquired vena cava obstructions after atrial switch operation using spiral computerized tomography and 3-dimensional reconstruction].

We report spiral-CT findings in adult patients with congenital abnormalities of the vena cava and systemic venous obstructions after atrial switch operation. Especially systemic venous obstruction is a well-known complication following Mustard procedure for transposition of the great arteries. The results demonstrate that computed tomography, particularly with the use of 3-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of abnormalities of the vena cava or of residua and sequelae after inflow correction for complete transposition. Demonstration of such abnormalities or obstructions after atrial switch operation is important, since the recognition and quantitation of caval anomalies by clinical techniques is unreliable and indeed often impossible. Imaging procedures such as spiral computed tomography are important for this purpose. Advantages of spiral computed tomography, particularly with 3-dimensional reconstruction, are discussed.

Adolescent↗
PubMed1997

Sequelae after modified Fontan operation: postoperative haemodynamic data and organ function.

OBJECTIVE: To investigate the specific sequelae of the Fontan operation, and particularly the potential sequelae of chronically elevated systemic venous pressure. DESIGN: A retrospective analysis of clinical and haemodynamic data and evaluation of organ function in 80 surviving patients undergoing modified Fontan operation for various forms of underlying functionally univentricular hearts. PATIENTS: 65 patients (81%) who had undergone a total cavopulmonary anastomosis and 15 an atriopulmonary anastomosis. Follow up ranged from 12 to 106 months (mean 54 (SD 23) months). RESULTS: 62 patients underwent postoperative cardiac catheterisation (mean systemic venous pressure 10.5 (2.5) mm Hg and cardiac index 3.1 (0.7) l/min/m2). Older age at operation was significantly correlated with both higher systemic venous pressure and lower cardiac index. Atrial arrhythmia was documented on Holter electrocardiogram in 17%. Protein losing enteropathy (with abnormal alpha 1-antitrypsin clearance) was found in 2/80 patients (2.5%). Ten patients had hypoproteinaemia, with a significantly higher incidence in patients after total cavopulmonary anastomosis and young age at operation. Liver function tests reflecting liver synthesis and metabolism were normal in all, whereas mild cholestasis was found in nearly 30%-predominantly in patients with a cardiac index of < 3 l/min/m2 (P = 0.045). Five patients (6.2%) developed atrial thrombosis. Coagulation factor analysis in 44 patients showed protein C deficiency in 11 (25%); laboratory signs of activation of the coagulation system were found in four of these (9%). None of the abnormal laboratory indices was significantly related to underlying cardiac malformation, postoperative systemic venous pressure, or follow up interval. CONCLUSIONS: A high proportion of clinically asymptomatic patients had abnormal laboratory findings on mid-term follow up. Detailed evaluation of organ function is necessary to detect the need for further diagnostic procedures before clinical symptoms develop.

Adolescent↗
PubMed1996

Isolated aortic coarctation in neonates and infants: results of resection and end-to-end anastomosis.

BACKGROUND: Operative resection is the treatment of choice for native aortic coarctation in most institutions. The ideal timing for elective repair is still a matter of debate. This study evaluated one institution's results with resection and end-to-end anastomosis in the first year of life. METHODS: Between January 1987 and December 1993, 46 neonates and infants with functionally isolated aortic coarctation underwent operative resection and end-to-end anastomosis. For the patients included in the study, all hospital records, catheterization data, and operative protocols were evaluated for retrospective analysis. To obtain valid follow-up information, all patients were systematically seen on an outpatient basis during 1994. RESULTS: After a mean follow-up of 49 +/- 24 months (range, 13 to 95 months), recoarctation (arm-leg blood pressure gradient > 20 mm Hg) occurred in 5 of 26 patients with neonatal operations (19%) and in 1 of 20 patients with operations in infancy (5%, p = not significant). Four of these 6 children with recoarctation needed reintervention. The other 2 patients had only mild recoarctation (gradients of 22 and 30 mm Hg, respectively) and were not treated. In all 6 patients, recoarctation was diagnosed within the first 6 months postoperatively. During the whole follow-up period, right arm systolic blood pressures slightly above the 90th percentile of normal developed in 11 of the patients (24%) (7 in the group with neonatal operation and 4 after operation in infancy; p = not significant). CONCLUSIONS: Resection with end-to-end anastomosis was shown to be an adequate therapeutic strategy for isolated aortic coarctation in neonates and infants. The results indicate that already beyond the neonatal age, there is a relatively low incidence of recoarctation.

Anastomosis, Surgical↗
PubMed1996

Modified Fontan operation in functionally univentricular hearts: preoperative risk factors and intermediate results.

BACKGROUND: The modified Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy. To eliminate the influence of different surgical strategies, only patients undergoing total cavopulmonary anastomosis were included in this retrospective study. METHODS: Seventy-two patients had been operated on at ages ranging from 7 to 219 months, with 29 patients younger than 4 years. Twenty-three patients had mean pulmonary artery pressures higher than 15 mm Hg, elevated pulmonary arteriolar resistances (> 3 U.m2), or elevated end-diastolic ventricular pressure (> 12 mm Hg). Associated systemic or pulmonary venous anomalies were present in 22 patients (30%), and atrioventricular valve incompetence was present in 21 patients (29%). RESULTS: The overall mortality rate was 9.7% (7/72). Variables with significant effects on postoperative mortality were associated systemic or pulmonary venous anomalies, atrioventricular valve incompetence, mean pulmonary artery pressure greater than 15 mm Hg, and prolonged cardiopulmonary bypass time. Postoperative morbidity resulted mainly from atrial arrhythmia (20%). Clinical signs of protein-losing enteropathy or atrial thrombi were rare (3% and 6%, respectively). Postoperative hemodynamic data from 48 surviving patients (74%) revealed a mean transpulmonary gradient of 6.3 mm Hg, systemic venous pressure greater than 12 mm Hg in only 10 patients, and cardiac index less than 3 L.min-1.m-2 in 18 patients. CONCLUSION: In a high proportion of hearts with complex univentricular anatomy, associated anomalies, and borderline hemodynamics, the Fontan operation can be performed as a total cavopulmonary anastomosis with acceptable intermediate postoperative morbidity and hemodynamic results.

Adolescent↗
PubMed1996

[Atrial flutter in young patients after corrective operation of congenital heart defects: therapy by high frequency catheter ablation].

UNLABELLED: Radiofrequency catheter ablation has been used successfully in adult patients for treatment of atrial flutter. Three young patients (mean age 14.6 years) with common type atrial flutter (n = 2) and uncommon type atrial flutter (n = 1) underwent electrophysiologic study. Cardiac diagnoses included tricuspid atresia after Fontan operation, status after closure of an atrial septal defect of secundum type, and status after surgical valvotomy for valvular pulmonary stenosis, respectively. Indication for ablation were syncopal episodes in one and presyncopal episodes in two patients. Flutter cycle length ranged from 280 to 320 ms. Right atrial endocardial mapping revealed areas with local electrograms preceding the onset of the flutter-P-wave by 70 to 80 ms. In these regions pace mapping was performed with the intention to produce an identical P-wave morphology and short stimulus to P-wave interval. Radiofrequency current application (500 kHz) with 30 W for 30 s at theses sites terminated atrial flutter and prevented reinduction in all three patients. No complications were observed. Follow-up (7 to 11 months) revealed recurrence of uncommon type of atrial flutter in one patient who previously had had common type. The remaining two patients are in stable sinus rhythm. CONCLUSION: Radiofrequency catheter ablation appears to be an effective treatment of atrial flutter after surgery for congenital heart defects in young patients.

Adolescent↗
PubMed1996

[Intermediate and long-term outcome after percutaneous balloon dilatation of valvular pulmonary stenoses in childhood].

Pulmonary balloon valvuloplasty was performed in 52 patients aged 7 days to 19 years (mean 5.5 years). Mean balloon/anulus ratio was 1.24. Mean right ventricular outflow tract (RVOT) systolic pressure gradient was 79.9 +/- 37.3 (x +/- SD) mm Hg before valvuloplasty and 37.2 +/- 29.6 mm Hg (p < 0.001) immediately after the procedure. 33 patients had residual RVOT-gradient < or = 36 (22 +/- 7) mm Hg, 19 patients had gradients > 36 (67.1 +/- 35.6) mm Hg. During intermediate follow-up (< 2 years) RVOT gradient assessed by catheterization or Doppler echocardiography improved without any additional intervention in 10/19 patients with RVOT gradient > 36 mm Hg early after valvuloplasty. 6/19 patients required additional reduction of RVOT gradient by repeated valvuloplasty (51 +/- 19 to 29 +/- 5 mm Hg; p < 0.01). 3/19 patients needed surgical resection of extremely thickened dysplastic valves. 49 patients had a mid- to long-term follow-up by echocardiography (mean 4.3, max. 9 years); the RVOT gradient decreased from 25.7 +/- 12.8 mm Hg during intermediate follow-up to 18.0 +/- 7.0 mm Hg during long-term follow-up. If pulmonary regurgitation was present after dilatation (n = 38), it was hemodynamically not significant and did not change during follow-up. Percutaneous balloon valvuloplasty was a safe and effective treatment for pulmonary valve stenosis in infancy and childhood. Long-term results confirm the value of this method.

Adolescent↗
PubMed1996

[Coronary sinus defects: rare form of interatrial communication].

UNLABELLED: A coronary sinus defect results in a communication of variable size between the coronary sinus (CS) and the left atrium (LA). It is in most cases associated with a persistent left superior vena cava (l-SVC) and an atrial septal defect of the coronary sinus type and often part of a more complex cardiac malformation. If the CS to LA fenestration is the single cardiac defect, this anomaly provides the basis of an unusual form of interatrial communication, which may cause diagnostic difficulties. Two patients with this rare anomaly are reported. A 9-year-old boy presented with history of mild cyanosis pronounced after physical activity (SaO2 < 90%) since the age of 3. Comprehensive pulmonary and cardiological diagnostic procedures in the referring hospital yielded normal findings. Finally, a 1-SVC draining into a mildly dilated coronary sinus could be demonstrated echocardiographically. Contrast echocardiography revealed a right-to-left-shunt at atrial level. Diagnosis of a partially unroofed coronary sinus was subsequently confirmed during cardiac catheterization. The second patient was primarily diagnosed echocardiographically to have an atrial septal defect of the primum type (ASD I) in the first year of life. After pulmonary vascular markings on chest-x-ray had increased, she was assigned to our hospital for cardiac catheterization. Slight arterial desaturation (SaO2 88%) was present due to a large l-SVC anomalously draining into the LA, the innominate vein was missing. The roof of the coronary sinus was predominantly absent. Combined with a large atrial septal defect of the coronary sinus type, this resulted in significant left-to-right-shunt and right-to-left-shunt at atrial level due to a pure unroofed coronary sinus. CONCLUSION: A coronary sinus defect with partial or complete unroofing of the CS and a persistent l-SVC should be considered in the differential diagnosis of an otherwise unexplained systemic desaturation and in patients with both left-to-right-shunt and right-to-left-shunt at atrial level.

Cardiac Catheterization↗
PubMed1995

Total cavopulmonary anastomosis in patients less than three years of age.

BACKGROUND: As young age at modified Fontan operation was thought to be a preoperative risk factor for poorer survival, we studied early and intermediate outcome in our young patient group. METHODS: Results in children less than 3 years of age (group I; n = 26; age range, 7 to 35 months) were compared with those in older patients (group II; n = 46; age range, 36 to 219 months). For both groups there was no significant difference with regard to preoperative pathology and hemodynamics. RESULTS: With an overall mortality of 9.7% (7/72) there was no significant difference for both groups. Group I (n = 23) and group II (n = 42) survivors did not differ with respect to early postoperative incidence of atrial dysrhythmias, duration and volume of pleural effusion, or incidence of reoperation. Results on intermediate follow-up (group I, 31 +/- 14 months; group II, 44 +/- 20 months) demonstrated a relatively low incidence of late atrial dysrhythmias (12.3%; 8/65). Late atrioventricular valve insufficiency was significantly more frequent in group I patients. CONCLUSIONS: With similar preoperative anatomic and hemodynamic parameters including 68% of patients with complex univentricular heart, total cavopulmonary anastomosis could be performed in patients less than 3 years of age with good clinical and hemodynamic results, as achieved in older patients.

Child, Preschool↗
PubMed1994

Atrial baffle procedures for complete transposition of the great arteries: natural course of sinus node dysfunction and risk factors for dysrhythmias and sudden death.

359 patients after the Mustard (275) or Senning (84) operations for transposition of the great arteries were followed-up for a mean of 103.7 (range 0.4 to 204) months. 259 patients had postoperative 24-h Holter recordings. In 129 Mustard children serial (mean 4.4) postoperative Holter recordings were available for evaluation. Criteria based on Holter and scalar electrocardiograms at normal sinus node functions were used for rhythm analysis. Postoperative dysrhythmias appeared in 70% of the patients: sinus node dysfunction in 62.8%, second or third degree atrioventricular block in 3.2%, sustained atrial tachycardia or atrial flutter in 4.5%, and significant ventricular arrhythmia (Lown 2-5) in 21.4%. The prevalence of sinus node dysfunction increased slightly from 50.8% during the first 2 postoperative years to 64.4% in patients more than 10 years postoperatively. Fifteen patients (4.2%) died suddenly during follow-up. By multivariate analysis severe tricuspid regurgitation and/or right ventricular dysfunction and uncontrolled supraventricular tachydysrhythmias were identified as the two significant risk factors for sudden death.

Adolescent↗
PubMed1993

[The automatic implantable cardioverter-defibrillator for prevention of sudden heart death in children and adolescents].

Little experience exists with the automatic implantable cardioverter-defibrillator in the pediatric population. Since 1990, an automatic implantable cardioverter defibrillator was implanted in four young patients (mean age 15.8 years, mean body weight 53.3 kg) with life-threatening ventricular tachyarrhythmias at our institution. In three patients, a cardiac anomaly was evident (dilated cardiomyopathy, status post Rastelli operation for complex transposition of the great arteries, status post atrial switch for transposition of the great arteries), the last patient had a normal cardiac anatomy. Indications for implantation were resuscitation from documented hypotensive ventricular tachycardia in one patient and recurrent syncope of suspected cardiac origin in the remaining three patients. At preimplantation electrophysiological study, all four patients had inducible ventricular tachycardia and/or ventricular fibrillation. At implantation of the cardioverter defibrillator in the operating theatre, the ventricular tachyarrhythmias were again induced and terminated reliably by the device. After a mean follow-up of 13 months, three of the four patients had appropriate discharges without syncope or resuscitation. The automatic implantable cardioverter-defibrillator appears to be a feasible and effective therapy also in pediatric patients for prevention of sudden cardiac death due to ventricular tachyarrhythmias.

Adolescent↗
PubMed1993

Coarctation of the pulmonary artery in neonates. Prevalence, diagnosis, and surgical treatment.

Stenosis or discontinuity of the central pulmonary artery at its bifurcation is often found in patients with congenital heart disease with intracardiac obstruction of the right side. The prevalence of this disease in autopsy cases is about 30%. The clinical prevalence has not been established yet because early diagnosis of this lesion is easily missed. Embryology suggests an etiologic similarity between coarctation of the aorta and this type of stenosis: both are caused by invasion of ductal tissue into the respective great artery. Therefore, the term coarctation of the pulmonary artery is appropriate. To recognize or exclude coarctation of the pulmonary artery, we examined left ventricular angiograms, aortograms, or both in the hepato-clavicular++ view in 25 consecutive neonates with severe arterial hypoxemia caused by right ventricular outflow tract obstruction. The prevalence of coarctation of the pulmonary artery was 36% (9/25). Fifteen neonates had two ventricles, and the other 10 had different types of univentricular heart. In eight cases no patent ductus arteriosus was found, and in none of these cases was coarctation of the pulmonary artery present. Conversely, in 9 of 17 neonates with a patent ductus arteriosus, coarctation of the pulmonary artery of different degree was demonstrated, even during prostaglandin E1 infusion in 7 neonates. With regard to surgical procedures, ductal tissue in the pulmonary artery wall should be completely removed. The aim is to establish adequate blood flow to both right and left pulmonary arteries. In this way unilateral hypoplasia of the arterial tree and consecutive growth of tortuous bronchial collaterals is prevented.

Abnormalities, Multiple↗