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D Schranz

Publications and source records attributed to D Schranz.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Heart transplantation in infancy: experiences at the Giessen Pediatric Heart Center].

From June 1988 to December 1996 heart transplantations were performed in 36 newborns and infants below one year of age. Diagnosis were hypoplastic left heart syndrome (n = 26), endocardial fibroelastosis (n = 4), cardiomyopathy (n = 3), and other complex congenital heart defects (n = 3). Mean waiting time for transplantation was 52 days, the mean donor-recipient bodyweight ratio was 1.8. Seven patients (19%) died after transplantation mainly within the first month after transplantation. The cumulative probability of survival is 79% in all patients. The influence of increasing experience is indicated when patients transplanted from 1988-1993 (n = 15) are compared with transplants from 1994-1996 (n = 21). The overall survival in the first group was 50%, whereas patients transplanted from 1994 showed a probability of survival of 92%. The 1-year survival rate in the later group was 100%. In 20 patients a total of 31 rejection episodes were observed. 2 infants died due to rejection. 71% of all rejections occurred during the first month after transplantation. Renal function was slightly impaired one year after transplantation in all patients without tendency for deterioration in the sequel. The somatic development is normal in nearly all infants and the quality of life is excellent. All infants live at home without any restrictions. Two patients, however, suffer from a neurologic deficit. Until now there is no evidence of coronary vascular disease or malignancy. Heart transplantation is in our opinion a reconsiderable alternative in the treatment of complex cardiac disease and cardiomyopathy in infants.

Cause of Death↗
PubMed1998

Clinical experience with heart transplantation in infants.

OBJECTIVE: Orthotopic heart transplantation has become an accepted therapeutic concept for adult patients with endstage heart disease. In newborns and infants this procedure is still a matter of discussion because of unknown long-term results and the lack of donor organs. METHODS: Since March 1988 we have performed 40 orthotopic heart transplantation in 39 infants who were from 1 to 280 days of age. Indications for transplantation included hypoplastic left-heart syndrome (n = 28), dilative cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and other complex structural anomalies (n = 3). The mean waiting period for transplantation was 53 days. A donor-recipient weight ratio up to 4.0 was accepted. Profound hypothermic circulatory arrest was used for graft implantation in all those patients who required extensive aortic arch reconstruction (71%). The initial immunomodulation was based on Cyclosporine, Azathioprine and Prednisolone. Patients who underwent transplantation during the first 6 weeks of life received a chronic single-drug therapy with Cyclosporine after 1 year. RESULTS: There were six peri-operative deaths caused by drug-resistant right-heart failure in three cases, humoral rejection (n = 1), CMV infection (n = 1) and multi organ failure (n = 1). One infant died late, due to rejection. The actuarial survival rate for the entire group is now 82%. There is a remarkable influence of increasing experience. Whereas six of 15 infants who had heart transplantation between 1988 and 1993 died early post-operatively (survival rate: 60%), only one late death occurred among 24 recipients in the period from 1994 to April 1997 (survival rate: 96%). Episodes of rejection occurred once or several times in about half of the patients in this series (48%). All surviving children are living at home in excellent condition. CONCLUSIONS: Heart transplantation during early infancy is a rational and durable therapy for heart diseases with irreversible myocardial failure or severe structural anomalies. The intermediate-term results have been encouraging in many centers, but more data must be accumulated to determine the sequelae of chronic immunosuppression. The lack of donor organs remains one of the major problems in pediatric heart transplantation.

Body Height↗
PubMed1998

Superior caval venous syndrome after atrial switch procedure: relief of complete venous obstruction by gradual angioplasty and placement of stents.

Superior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective. The purpose of our study therefore, was to assess immediate and medium term results of inserting stents subsequent to gradual balloon enlargement of acquired atresia of the intraatrial baffle in patients who had undergone an atrial switch operation. We investigated five patients with complete obstruction of the superior caval venous pathway at perforation of the atretic segment was achieved using a guide wire technique. The procedure was successful in all patients. Gradual angioplasty was performed and intravascular stents were implanted. The pressure in the superior caval vein dropped to normal values, symptoms improved, and the patency of the newly created venoatrial communication was proven at mid-term follow-up. Thus critical obstructions at the superior caval venous pathway after the Mustard procedure can be reopened by interventional catheterization. Implantation of balloon-expandable intravascular stents is safe and effective in the acute relief of the obstructions, but careful long-term follow-up is mandatory.

Adolescent↗
PubMed1998

Percutaneous closure of secundum atrial septal defect with a new self centering device ("angel wings").

OBJECTIVE: To investigate the safety, efficacy, and clinical application of a new self centering device ("angel wings") for closure of secundum atrial septal defects (ASD II) and persistent foramen ovale in all age groups. DESIGN: Multicentre, prospective, nonrandomised study. PATIENTS INCLUSION CRITERIA: defects with an occlusive diameter of < or = 20 mm and a surrounding rim of > 4 mm; body weight > 10 kg; and an indication for surgical closure of secundum atrial septal defect. Additionally, there were compassionate indications for closure in patients with persistent foramen ovale. INTERVENTIONS: Defects were closed by a transcatheter device consisting of two square frames made of superelastic nitinol wire. The frames are covered by elastic polyester fabric, which is sewn together at a central circle. All procedures, except for three interventions that were carried out under sedation, were performed under general anaesthesia using transoesophageal echocardiography and fluoroscopy to monitor intervention. RESULTS: Closure was attempted in 75 (71%) of 105 patients. An ASD II was present in 35 children and 15 adults. A persistent foramen ovale was present in 25 adults with suspected paradoxical embolism. Transcatheter closure was unsuccessful in three children and crossover to surgery was required. Residual shunts were found in 20 patients (27%) immediately after the procedure. A transient atrioventricular third degree block occurred in three patients (4%) and the right atrial disk was not fully deployed in three. A minor shunt (< 3 mm) was present in only three (4%) of 72 patients during follow up of 1-17 months. Blood clots on the right atrial disks in two patients (one required lysis) were seen during follow up transoesophageal echocardiography. Serious complications demanding surgical removal of the device occurred in three patients. One patient had haemopericardial tamponade because of an aortic lesion. Left atrial thrombus formation due to an unfolded right atrial disk was found in a second patient and dislodgement of the left atrial disk resulted in a large residual shunt in a third. CONCLUSIONS: Percutaneous closure of a central ASD with a diameter < or = 20 mm in paediatric and adult patients is feasible and effective with this new device. It is a promising alternative to surgical closure. Modifications of the design, however, seem to be mandatory as 4% of patients developed serious complications.

Adolescent↗
PubMed1997

Evaluation of gastric intramucosal pH during and after pediatric cardiac surgery.

OBJECTIVES: In adult patients, intramucosal pH (pHi) has been advocated to detect postoperative complications. The purpose of our study was to evaluate this technique in pediatric patients during and after cardiac surgery. METHODS: Thirty-five infants (age: 5 days to 15 years, median 1.8 years; and weight: 3.2-32 kg, median 9.8 kg) were studied. pHi was measured before cardiopulmonary bypass (CPB), after 30 min of CPB, prior to weaning off CPB, at intensive care unit arrival, and 6, 12, 24, 48 and 72 h after surgery. RESULTS: There were no complications related to the tonometer. A pathologically low pHi < 7.32 was found during surgery in less than 17%, at intensive care unit arrival in 83% and after 48 h in 18%. pHi values were lower (P < 0.05) at intensive care unit arrival (7.25 +/- 0.08) and after 6 h (7.28 +/- 0.09) than afterwards. pHi correlated with arterial pH (r = 0.66), central-peripheral temperature difference (r = -0.36), lactate (r = -0.32) and central venous pressure (r = -0.21). Patients after a Fontan procedure had postoperatively a lower pHi than after other operations (P < 0.05). None of the patients died or developed organ failure. Six patients had signs of organ dysfunction. Their pHi (median 7.23, range 7.14-7.28) could not differentiate them from the other patients. CONCLUSIONS: With current equipment, tonometry cannot be recommended for the management of pediatric patients after cardiac surgery. However, as a semi-invasive method tonometry deserves further evaluation.

Adolescent↗
PubMed1997

[Oral gentic syndromes causing dental anomalies].

The author briefly described those genetic syndromes that are accompanied by tooth or dental arch anomalies. Some of these can be corrected by prosthetic means. These anomalies can be indications for prosthetic treatment.

Dental Prosthesis↗
PubMed1996

Gradual angioplasty and stent implantation to treat complete superior vena cava occlusion after Mustard procedure.

A 16-year-old male was admitted with complete occlusion of the superior vena cava pathway 14 years after Mustard procedure for transposition of the great arteries. From a left subclavian vein approach, the atretic vein segment was perforated using a straight guidewire, and was followed by sequential balloon dilation. Implantation of a 30 mm Palmaz-stent through a femoral vein approach resulted in a widely patent channel of the vena cava superior into the systemic venous atrium.

Adolescent↗
PubMed1996

Continuous measurement of cardiac output by the Fick principle in infants and children: comparison with the thermodilution method.

OBJECTIVE: To compare a system that continuously monitors cardiac output by the Fick principle with measurements by the thermodilution technique in pediatric patients. DESIGN: Prospective direct comparison of the above two techniques. SETTING: Pediatric intensive care unit of a university hospital. PATIENTS: 25 infants and children, aged 1 week to 17 years (median 10 months), who had undergone open heart surgery were studied. Only patients without an endotracheal tube leak and without a residual shunt were included. METHODS: The system based on the Fick principle uses measurements of oxygen consumption taken by a metabolic monitor and of arterial and mixed venous oxygen saturation taken by pulse- and fiberoptic oximetry to calculate cardiac output every 20s. INTERVENTIONS: In every patient one pair of measurements was taken. Continuous Fick and thermodilution cardiac output measurements were performed simultaneously, with the examiners remaining ignorant of the results of the other method. RESULTS: Cardiac output measurements ranged from 0.21 to 4.55 l/min. A good correlation coefficient was found: r2 = 0.98; P < 0.001; SEE = 0.41 l/min. The bias is absolute values and in percent of average cardiac output was - 0.05 l/min or - 4.4% with a precision of 0.32 l/min or 21.3% at 2 SD, respectively. The difference was most marked in a neonate with low cardiac output. CONCLUSION: Continuous measurement of cardiac output by the Fick principle offers a convenient method for the hemodynamic monitoring of unstable infants and children.

Adolescent↗
PubMed1996

Purpura fulminans in severe congenital protein C deficiency: monitoring of treatment with protein C concentrate.

UNLABELLED: This report describes the successful use of protein C concentrate to treat severe purpura fulminans in a homozygous protein C-deficient infant for 8 months until oral anticoagulation was initiated. While fresh frozen plasma was previously used in such cases to replace protein C in the acute phase, the availability of a monoclonal antibody purified protein C concentrate now allows specific replacement of protein C, avoiding problems of fluid overload. An occlusive-hydrocolloid bandage proved to be effective in local treatment of skin lesions. D-dimer, fibrin monomer, thrombin-antithrombin complex and prothrombin fragment 1 + 2 were useful markers in monitoring and optimizing protein C replacement therapy. CONCLUSION: Diagnosis of protein C deficiency should be considered in a newborn with purpura fulminans. Early diagnosis and adequate replacement therapy is life-saving. Today, administration of protein C is the acute as well as long-term therapy of choice.

Antibodies, Monoclonal↗
PubMed1996

[Intravascular stents for treatment of systemic venous obstructions].

Two young adults, 16 and 18 years of age were admitted with complete occlusion of the superior vena cava pathway following previous Mustard procedure. In both patients, perforation of complete occluded vein segment using a straight guidewire and subsequent sequential enlargement by balloon dilation were successfully performed by a left subclavian vein approach. Implantation of a 30 mm Palmaz-stent resulted in a widely patent channel of the vena cava superior into the systemic venous atrium. In short-term follow-up of 3 months both stents remained patent. Repeat dilation of the stents was performed after 3 months. A third patient with systemic venous obstruction was a 3-year old child who suffered low cardiac output following modified Fontan operation caused by a narrowing of the superior vena cava to right pulmonary artery connection and stenosis of distal left pulmonary artery. The severe hemodynamic problem was resolved by implantation of Palmaz-stents in the two separate obstructions. At recatheterization 6 months later, both stents were patent without any residual pressure gradient.

Adolescent↗
PubMed1996

[Surgical closure of atrial septum defects via right-sided thoracotomy. Value with reference to the development of interventricular closure techniques].

Surgical closure of atrial septal defect is a safe and effective procedure with low morbidity and mortality. A right anterior thoracotomy approach is a suitable alternative to that through a median sternotomy and provides superior cosmetic results. Thirty patients at the age of 1 year, 3 months to 49 years underwent repair of atrial septal defects through a right thoracotomy. Twenty-four patients had secundum, three ostium primum, two sinus venosus defect, and one patient had Scimitar's syndrome. Details of the surgical procedure on cardiopulmonary bypass are presented. There was no operative or late mortality, and no morbidity directly related to the alternative approach. All patients or their parents considered the cosmetic result fair or satisfying. The following paper reflects our experience with the thoracotomy approach for repair of atrial septal defects, as well as a critical review of new developments in interventional ASD occlusion techniques.

Adolescent↗
PubMed1995

Transcatheter radiofrequency perforation and stent implantation for palliation of pulmonary atresia in a 3060-g infant.

In a 3060-g infant with fibromuscular pulmonary atresia an open right ventricular outflow tract was created by means of interventional cardiological methods. Following two inadvertent perforations without sequelae or clinical symptoms, radiofrequency perforation and subsequent balloon dilatation were successfully performed. The implantation of a Palmaz iliac stent led to a predictable communication between the right ventricle and pulmonary artery.

Cardiac Catheterization↗
PubMed1995

Evaluation of the pulse wave arrival time as a marker for blood pressure changes in critically ill infants and children.

OBJECTIVE: Pulse arrival time (PAT), which is the interval between the R wave of the electrocardiogram (ECG) and the arrival of the pulse wave peripherally, has been reported to be suitable for use as an indirect measure of blood pressure change. The purpose of this study was to evaluate, in critically ill infants and children, the degree to which 1/PAT covaries with systolic, diastolic, and mean blood pressure, as well as heart rate. METHODS: A laboratory device was used to calculate PAT in real time from the ECG and the plethysmographic curve of pulse oximetry used for routine monitoring. Calculated PAT and corresponding blood pressures and heart rate were stored on hard disk. A total of 15 critically ill patients, aged 6 days to 16 years, weighing 3 to 80 kg, were studied. RESULTS: In all patients, one period of 11,000 to 36,000 beats could be evaluated. Mean correlation coefficients were best for systolic blood pressure (r = 0.73), followed by mean blood pressure (r = 0.68) and diastolic blood pressure (r = 0.61), and, finally, heart rate (r = 0.52). In 7 patients, the correlation coefficient for systolic blood pressure was > 0.9; but, in 4 patients, it was < 0.5. CONCLUSIONS: We conclude that the correlation between 1/PAT and systolic blood pressure is not strong enough to serve as a marker for blood pressure changes in critically ill infants and children. This may be due to changes of the preejection period, which is part of the PAT.

Adolescent↗
PubMed1995

Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses.

UNLABELLED: In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%-90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respectively, with peak velocities exceeding 2.5 m/s. Of the 84 patients with satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our patients. None of the control persons showed mitral or aortic regurgitation. CONCLUSION: Aortic and mitral regurgitation are more frequent in patients with MPS than previously thought and that therefore these patients should have regular colour Doppler flow mapping and antibiotic prophylaxis when required.

Adolescent↗
PubMed1995

Norepinephrine, enoximone, and nitric oxide for treatment of myocardial stunning and pulmonary hypertension in a newborn with diaphragmatic hernia.

A newborn with right diaphragmatic hernia suffered myocardial stunning and suprasystemic pulmonary hypertension secondary to postpartal asphyxia. In addition to conventional therapy, norepinephrine, enoximone, and inhalational nitric oxide were successfully used. Norepinephrine improved myocardial perfusion pressure; the addition of enoximone, a phosphodiesterase-inhibitor, to beta-adrenergic agents increased cardiac performance. with decreasing concentrations of inhalational nitric oxide, severe pulmonary hypertension resolved after a few days, suggesting that transient endothelial dysfunction was partially responsible for pulmonary vasoconstriction in the newborn with congenital diaphragmatic hernia.

Enoximone↗
PubMed1995

Age-dependent changes in the beta-adrenoceptor-G-protein(s)-adenylyl cyclase system in human right atrium.

With increasing age, cardiac beta-adrenoceptor function decreases. To study possible mechanisms underlying this process, we assessed in right atrial appendages from 52 patients of different ages (group A, < 20 years, mean age 3.7 +/- 1.0 years, n = 20; group B, 20-50 years, mean age, 37.9 +/- 2.3 years, n = 9; group C, > 50 years, mean age 66.1 +/- 1.5 years, n = 23) without apparent heart failure who were undergoing open heart surgery beta-adrenoceptor number and subtype distribution (by (-)-[125I]-iodocyanopindolol [ICYP] binding), adenylyl cyclase activity, and Gs- and Gi-protein alpha-subunits (by quantitative Western blotting). beta-Adrenoceptor number in the three groups was not significantly different; in contrast, basal, 10 microM GTP-, 100 microM isoprenaline (ISO), 10 mM NaF-, 100 microM forskolin-, and 10 mM Mn(2+)-stimulated adenylyl cyclase activity was significantly higher in group A than in group B and was further decreased in group C. Similarly, 100 microM terbutaline-, 100 microM histamine-, and 100 microM 5-HT-stimulated adenylyl cyclase activity significantly decreased from group A to group C. Moreover, all these adenylyl cyclase parameters were significantly negatively correlated with the age of the patients. Although Gs alpha was not altered, Gi alpha in group C was significantly higher than in group A; moreover, there was a weak but significant positive correlation between Gi alpha and the age of the patients. We conclude that an impairment of the activity of the catalytic unit of adenylyl cyclase is involved in the decrease in cardiac beta-adrenoceptor function with age; an increase in Gi alpha might contribute further to the reduced beta-adrenoceptor function.

Adenylyl Cyclases↗
PubMed1995

Independent right lung high frequency and left lung conventional ventilation in the management of severe air leak during ARDS.

Pulmonary air leaks are one of the most common problems in patients with the adult respiratory distress syndrome, ARDS. We report what we believe to be the first case in which unilateral high-frequency ventilation combined with contralateral conventional positive pressure ventilation has been used successfully to manage severe air leak in an infant with ARDS.

Bronchial Fistula↗