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Biomedical subjects

R Hetzer

Publications and source records attributed to R Hetzer.

At least 73 records · Page 4Linked to original sources
PubMed2001

Capillary leak syndrome in children who undergo cardiopulmonary bypass: clinical outcome in comparison with complement activation and C1 inhibitor.

OBJECTIVES: Capillary leak syndrome (CLS) is associated with significantly increased morbidity and occurs after cardiopulmonary bypass in children with congenital heart disease. We investigated the early clinical parameters that predict the development of CLS and examined the relationship between the presence of CLS and complement and contact activation and C1 esterase inhibitor (C1-INH) during and after bypass. DESIGN: In this prospective study we took serial serological measurements of the complement and contact system and C1-INH in a cohort of 27 infants before, during, and up to 96 h after open-heart surgery. RESULTS: Complement and contact activation and a decrease in C1-INH were measured in all infants during and after CPB. Ten infants developed CLS postoperatively. Younger age and longer bypass time were strongly correlated to the development of CLS. No relationship was found between the degree of hypothermia, weight, gender, or cross-clamp time. C1-INH concentration and activity were lower peri- and postoperatively in the CLS group. Infants with CLS had a more pronounced postoperative increase in the C5a and C3a levels, higher postoperative level of factor XIIa, and lower prekallikrein activity than those without CLS. CONCLUSION: Contact and complement activation occurs during cardiopulmonary bypass and contributes to CLS more frequently in infants of a younger age and with a prolonged bypass time. This activation and decrease in C1-INH was strongly expressed in the CLS group, and therefore early substitution of C1-INH may prevent CLS after open-heart surgery in high-risk infants.

Capillary Leak Syndrome↗
PubMed2001

[Congenital coronary fistulas: the most frequent congenital coronary anomaly].

Congenital arteriovenous coronary fistulae are a rare condition of a direct communication between a coronary artery and one of the cardiac chambers, the coronary sinus, the superior vena cava or the pulmonary artery. In most instances the diagnosis is made during heart catheterization for coronary or congenital heart disease. Whether congenital coronary artery fistulae should be treated by transcatheter intervention or surgery and in which patients fistula closure should be performed is controversial. This report summarizes our experience of the surgical treatment of congenital arteriovenous coronary fistulae in 14 patients at the Deutsches Herzzentrum Berlin between March 1988 and April 1997. There were seven females and seven males aged from 3 to 67 years (mean 47 years). We analyzed the symptomatic status (NYHA class) preoperatively and in the late outcome, the preoperative angiographic data and the surgical techniques. The right coronary artery was affected in six, the left in six, and both arteries in two cases. The fistulae drained into the pulmonary artery in eight cases, into the superior vena cava and into the right atrium in two cases, and into the right ventricle and into the coronary sinus once. Fistula closure was unsuccessfully attempted interventionally in two patients and surgically in one patient in another institution. Twelve of the patients exhibited additional cardiac disease requiring surgery: seven cases presented additional coronary artery disease, one mitral valve disease, one persistent ductus arteriosus, one an aneurysm of the right coronary artery, and two an atrial septal defect. We performed fistula closure either by ligating or transsecting the fistula as well as by closure of the fistula's drainage opening. Surgery and postoperative courses were uneventful in all patients. Most of the patients (93%) were in good clinical condition (NYHA I-II) after a mean follow-up period of 6.6 years (range 3-11). Fistula closure should be performed in patients who are symptomatic or who have a hemodynamic relevant shunt. In asymptomatic patients and small left-to-right shunt, fistula closure should also be performed to prevent later complications. Surgical fistula closure should be employed in patients with larger and more complex fistulae, especially if interventional therapy failed, and for patients with additional cardiac conditions that necessitate surgery.

Adolescent↗
PubMed2001

[DDD-pacemaker implantation in a 7-week-old infant: epicardial electrodes in intrapleural position].

Permanent pacing for complete heart block in newborns and small infants is a challenge concerning the mismatch between body size and pacemaker dimensions. As the abdominal position of the large pacemaker carries a considerable risk of healing disturbances, the intrapleural position of single chamber pacemakers is accepted as well. We report about a 7 week old infant with congestive heart failure due to a complete atrioventricular septal defect. The immature valve tissue led to recurrent, severe mitral valve incompetence and necessitated several valvuloplasty procedures and finally the implantation of a mechanical prothesis. Because of a postoperative complete heart block, a dual chamber pacemaker with epicardial steroid-eluting bipolar leads was implanted without specific fixation into the right pleural cavity during delayed sternal closure to ensure atrioventricular synchrony. Despite the capacious implant, adverse effects on hemodynamics and pulmonary function were not observed and the infant thrived normally. The DDD function of the pacemaker allowed the utilization of the infant's complete heart rate spectrum without restrictions due to the technically limited upper tracking rate of 180 bpm.

Age Factors↗
PubMed2001

Early experience with extracardiac Fontan operation.

BACKGROUND: The extracardiac Fontan (ECFO) operation has gained more widespread use over last decade. In this article we analyze our early experience with ECFO. METHODS: Thirty-three patients underwent ECFO at median age 4.1 years. Normothermic perfusion with the heart beating was used in 24 patients and cardioplegia in 7. Fenestration was not performed in the last 16 patients who underwent surgery without cardioplegia. RESULTS: The hospital mortality was 6% (2 patients). Necessity or duration of inotropic support, duration of mechanical ventilation, intensive care unit and hospital stay, and incidence of prolonged pleural effusions of patients operated on without cardioplegia were shorter and the rate of complications in these patients was lower than in patients who underwent cardioplegia. CONCLUSIONS: Our preliminary experience shows that ECFO can be performed in many patients with normothermic cardiopulmonary bypass and without cardioplegia and fenestration. Avoidance of cardioplegia seems to decrease postoperative morbidity. Incidence of early postoperative arrhythmias is low. Despite encouraging early results, longer follow-up is necessary to prove the real advantages of ECFO.

Adolescent↗
PubMed2001

Development of pulmonary arteries after central aortopulmonary shunt in newborns.

BACKGROUND: Central shunt (CS) is frequently used to treat diminished pulmonary blood flow in newborns. We analyzed the impact of CS on the growth of the pulmonary arteries (PAs). METHODS: Twenty-two consecutive newborns underwent a CS procedure. In 15 newborns the preoperative angiograms and angiograms taken before undergoing anatomic or hemodynamic correction procedures were analyzed. The patients were divided retrospectively into two groups by the size of the PA in the preoperative angiogram: group I, patients with PAs more than 4 mm (n = 10), group II, PAs 4 mm or less (n = 5). To compare the development of the PAs in the groups, the Nakata index, McGoon ratio, and lower lobe indices were calculated from angiograms. RESULTS: The indices were significantly higher in group I before CS, but no differences was found between the groups before anatomic or hemodynamic correction. The postoperative Nakata indices and the McGoon ratios in the groups were higher when compared with preoperative values (group I, p = 0.037 and p = 0.013; group II, p = 0.043 and p = 0.043, respectively). The significant increase of the lower lobe indices only in group II (p = 0.043) suggests faster growth of the PA in this group. CONCLUSIONS: Optimal diameters of the CS promote growth of the PAs, which was confirmed by the increased Nakata and McGoon indices. The benefit in smaller PAs is greater.

Anastomosis, Surgical↗
PubMed2001

Bridging-to-recovery.

BACKGROUND: Patients with end-stage heart failure placed on a cardiac assist device show at least some degree of improvement of cardiac function. In a subgroup of selected patients, some hearts recovered considerable function. In these patients the device was removed and cardiac transplantation was no longer necessary. We report our long-term experience with these weaned patients. METHODS: As of today, 512 cardiac assist devices of various types (Berlin Heart, Berlin, Germany; Novacor, World Heart, Ottawa, Ontario, Canada; TCI, ThermoCardio Systems, Inc, Woburn, MA; DeBakey, Micromed Technology Inc, Houston, TX) were implanted in patients with end-stage heart failure in our institution. Of these, 95 patients belonged to a subgroup of patients with nonischemic, idiopathic, dilated cardiomyopathy who were implanted with a left ventricular support system (Novacor 84, TCI 10, Berlin Heart 1) between 1994 and 2000. All were routinely examined by echocardiography for improvement of cardiac function. The left ventricular diameter in diastole (LVIDd) and left ventricular ejection fraction (LVEF) served as the main parameters to assess changes in cardiac performance. Under the conditions of a running device, an LVIDd below 60 mm and an LVEF above 40% were the criteria to do further echocardiographic studies when the pump was turned off for up to 20 minutes. RESULTS: Twenty-eight patients (26 men, 2 women; ages 18 to 64 yrs; history of heart failure, 1 to 17 yrs) fulfilled the criteria of improved cardiac performance and were weaned from the device. Since then, 16 patients have continued "normal" heart function with follow-up times ranging from 1 month to 5.5 years (group B). Three patients died of noncardiac causes (group C). Eight patients were transplanted from 1 to 17 months later and one died on the waiting list (group A). Statistically significant differences between groups A and B were calculated for the duration of heart failure (9 versus 2 years, p = 0.0002). Differences in LVIDd before removal of the device (57 versus 51 mm, p = 0.0420), LVEF after 2 months of unloading (30 versus 49%, p = 0.0300), and LVEF preexplantation (43 versus 52%, p = 0.0001) were significant. Overall, 17% of the cohort of 95 patients were weaned successfully. CONCLUSIONS: Weaning from cardiac assist devices is feasible for selected patients; it saves donor hearts and is preferred to cardiac transplantation. However, as of today no reliable parameter predicts outcome after weaning and none determines the possibility of device removal before implantation in advance.

Adolescent↗
PubMed2001

Wound-healing disturbances after vein harvesting for CABG: a randomized trial to compare the minimally invasive direct vision and traditional approaches.

BACKGROUND: Harvesting the great saphenous vein for coronary artery bypass grafting is often associated with complications in wound healing, insufficient cosmetic results, and delay in mobilization of the patients. The aim of this study was to compare the results of our minimally invasive technique with the traditional method. METHODS: We report our experience of minimally invasive direct vision harvesting the great saphenous vein with the Aesculap retractor system (Aesculap AG Co KG, Tutlingen, Germany) by performing 3 (to 5) small cutaneous incisions. We scheduled 255 patients for elective coronary artery bypass grafting prospectively randomized to undergo vein harvesting by either the minimally invasive technique (group A: n = 128; age range, 68.2 +/- 9.1 years; male, 53.1%) or by the traditional technique (group B; n = 127; age range, 66.1 +/- 8.3 years; male, 62.9%). We classified and defined leg-wound healing disorders in terms of mild, moderate, and severe wound-healing disturbances. RESULTS: Between group A and B there were no differences with the risk stratification before operation, length of vein being harvested, or total operation time. The time for minimally invasive harvesting of the great saphenous vein was slightly increased. Severe leg-wound healing disorders occurred in 4 of 128 patients of group A (3.1%) versus 12 of 127 patients of group B (9.4%) with significant difference (p = 0.042). CONCLUSIONS: Minimally invasive direct vision harvesting the great saphenous vein is an attractive alternative to the traditional open-harvesting technique. In our trial this procedure resulted in fewer wound complications and showed a much better cosmetic outcome. The total operation time was not increased by using the minimally invasive technique.

Aged↗
PubMed2001

Intraoperative radiofrequency maze ablation for atrial fibrillation: the Berlin modification.

BACKGROUND: The Cox-maze procedure combined with an operation for organic heart disease is highly successful in the elimination of chronic atrial fibrillation. However, it prolongs significantly the aortic cross-clamp and operating time. In this study, a simplified left atrial maze procedure, which is a short procedure performed using a surgical radiofrequency ablation probe, is added to elective open heart procedures in patients with atrial fibrillation. METHODS: Forty-eight adults with atrial fibrillation (duration, 6 months to 36 years) underwent elective open heart operations (isolated valve procedures or coronary artery bypass grafting, n = 27 patients; combined procedures, n = 21 patients) combined with intraoperative radiofrequency ablation of the left atrium. The postoperative follow-up period ranged from 1 to 11 months (mean, 4 months). Possible predictors for persistent postoperative atrial fibrillation were determined among 40 variables by univariate and multivariate analyses. RESULTS: Intraoperative radiofrequency ablation prolonged the aortic cross-clamp time for 6 to 14 minutes (mean, 11 minutes). Freedom from atrial fibrillation was 100% intraoperatively, 25% at 1 week after operation (12 of 48 patients), 59% at 1 month postoperatively (16 of 27 patients), 64% at 3 months postoperatively (16 of 25 patients), and 92% at 6 months postoperatively (12 of 13 patients). The only predictor of postoperative atrial fibrillation was the presence of coronary artery disease (odds ratio, 7.5; 80% confidence interval, 2.24-25.13). CONCLUSIONS: Intraoperative radiofrequency ablation of the left atrium combined with an operation for organic heart disease effectively eliminates atrial fibrillation without significant prolongation of the aortic cross-clamp and operative time. The presence of coronary artery disease decreases the success rate during the first 6 postoperative months.

Adult↗
PubMed2001

Benefits of surgical repair of coarctation of the aorta in patients older than 50 years.

BACKGROUND: Most patients with unrepaired coarctation of the aorta die before the age of 50 years. In patients who present at an older age, the indications for surgical treatment are controversial because the benefits of operating are unclear. METHODS: At follow-up investigation from 0.5 to 11.5 years (mean, 4 years) after primary surgical correction of coarctation in 15 patients aged 50 to 63 years (mean, 54 years), we analyzed the preoperative and postoperative complications, symptoms, need for antihypertensive drugs, and blood pressure at rest and during exercise. RESULTS: Preoperatively no patient had normal blood pressure at rest despite combined antihypertensive medication. There was no significant mortality or morbidity after repair. At follow-up examination only 3 patients had at rest mild hypertension, the other 12 patients were normotensive. Of the 11 tested patients, 8 displayed systolic arterial hypertension during exercise. CONCLUSIONS: Surgical correction of coarctation can be performed after the age of 50 years with low surgical risk. Operation reduces systolic hypertension at rest and permits more effective medical treatment. Despite persistence of the hypertension during exercise, symptomatic improvement occurs in most patients.

Antihypertensive Agents↗
PubMed2001

Decreased oxidative stress in patients with idiopathic dilated cardiomyopathy one year after immunoglobulin adsorption.

OBJECTIVES: In a substudy to a recently reported investigation that demonstrated the benefit of immunoglobulin adsorption (immunoadsorption) for patients with idiopathic dilated cardiomyopathy (IDC), we tested whether this benefit is associated with a reduction of oxidative stress. BACKGROUND: The progression of cardiomyopathy is believed to be related to the increase of oxidative stress. Therefore, reduction of oxidative stress could be one of the effects of immunoadsorption for improvement of cardiac performance and clinical status. METHODS: Plasma markers for oxidative stress-thiobarbituric acid-reactive substances (TBARS), lipid peroxides (LPO), anti-oxidized low-density lipoprotein-autoantibodies (anti-oxLDL-AB), thiol groups and vitamin E-were compared in 31 patients, of whom 16 underwent immunoadsorption and 15 received conventional treatment (controls). All patients received a daily supplement of vitamins, minerals and trace elements. RESULTS: After one year, TBARS (p = 0.026), LPO (p = 0.026) and anti-oxLD

Adsorption↗
PubMed2001

Spectrum of clinical phenotypes and gene variants in cardiac myosin-binding protein C mutation carriers with hypertrophic cardiomyopathy.

OBJECTIVES: We studied the clinical and genetic features of hypertrophic cardiomyopathy (HCM) caused by mutations in the myosin-binding protein C gene (MYBPC3) in 110 consecutive, unrelated patients and family members of European descent. BACKGROUND: Mutations in the MYBPC3 gene represent the cause of HCM in approximately 15% of familial cases. MYBPC3 mutations were reported to include mainly nonsense versus missense mutations and to be characterized by a delayed onset and benign clinical course of the disease in Japanese and French families. We investigated the features that characterize MYBPC3 variants in a large, unrelated cohort of consecutive patients. METHODS: The MYBPC3 gene was screened by single-strand conformational polymorphism analysis and sequencing. The clinical phenotypes were analyzed using rest and 24-h electrocardiography, electrophysiology, two-dimensional and Doppler echocardiography and angiography. RESULTS: We identified 13 mutations in the MYBPC3 gene: one nonsense, four missense and three splicing mutations and five small deletions and insertions. Of these, 11 were novel, and two were probably founder mutations. Patients with MYBPC3 mutations presented a broad range of phenotypes. In general, the 16 carriers of protein truncations had a tendency toward earlier disease manifestations (33 +/- 13 vs. 48 +/- 9 years; p = 0.06) and more frequently needed invasive procedures (septal ablation or cardioverter-defibrillator implantation) compared with the 9 carriers of missense mutations or in-frame deletions (12/16 vs. 1/9 patients; p < 0.01). CONCLUSIONS: Multiple mutations, which include missense, nonsense and splicing mutations, as well as small deletions and insertions, occur in the MYBPC3 gene. Protein truncation mutations seem to cause a more severe disease phenotype than missense mutations or in-frame deletions.

Adolescent↗
PubMed2001

Decreased plasma concentration of brain natriuretic peptide as a potential indicator of cardiac recovery in patients supported by mechanical circulatory assist systems.

OBJECTIVES: We sought to investigate the relationship between the plasma concentration of brain natriuretic peptide (BNP), echocardiographic findings and the clinical outcome of patients supported with ventricular assist devices (VADs) to determine the role of BNP as a predictor for cardiac recovery. BACKGROUND: Ventricular unloading in patients with end-stage heart failure supported by VADs may lead to myocardial recovery. The BNP is produced in the myocardium in response to chronic volume overload, but the effects on it of ventricular unloading by VADs are largely unknown. METHODS: Twenty-one patients diagnosed with nonischemic cardiomyopathy and supported by VADs were evaluated for echocardiographic data and blood chemistry including BNP. They were divided into patients who died while on mechanical support (group I; n = 9), patients who were transplanted (group II; n = 8) and patients who were successfully weaned off the system and did not require transplantation (group III; n = 4). RESULTS: Brain natriuretic peptide plasma concentrations decreased significantly after initiation of mechanical circulatory support (p = 0.017). Furthermore, the changes in BNP plasma concentrations showed a faster decrease to normal levels within the first week after implantation of the VAD in patients who were weaned off the system (group III) compared to patients in group I and group II. CONCLUSIONS: This study shows that ventricular unloading with VADs decreases BNP plasma concentrations in patients who suffer from end-stage heart failure. Furthermore, we hypothesize that an early decrease of BNP plasma concentration may be indicative of recovery of ventricular function during mechanical circulatory support.

Adolescent↗
PubMed2001

Cold continuous antegrade blood cardioplegia: high versus low hematocrit.

OBJECTIVE: Cold continuous antegrade blood cardioplegia (CCABCP) is used with different hematocrit values. We investigated the consequences of CCABCP with low hematocrit (LH: 20-25%) versus high hematocrit (HH: 40-45%). METHODS: Anesthetized open chest pigs (25 kg) were placed on cardiopulmonary bypass (CPB). The hearts were arrested for 30 min by 6 degrees C CCABCP with either LH or HH (n=8, each): After an initial 3 min application of high potassium (20 mEq) BCP the hearts were arrested for subsequent 27 min by normokalemic 6 degrees C cold blood delivered continuously antegradely. Thereafter the hearts underwent perfusion with warm systemic blood for an additional 30 min on CPB. Biochemical cardiac data (MVO(2) (ml min(-1)100 g(-1)), release of creatine kinase (CK; units min(-1)100 g(-1))) and lactate (mg min(-1)100 g(-1))) and the coronary vascular resistance index (CVRI (mmHg ml(-1)ming)) were measured during CPB. Total tissue water content (%) and left and right ventricular stroke work indices (LV-and RV-SWI (g m kg(-1))) were assessed 30 min after discontinuation of CPB and compared to pre-CPB controls. RESULTS: The hearts of the LH group had no biochemical or functional disturbance. The HH group showed marked CK leakage (0.6+/-0.2* vs. 0.1+/-0.1, *P<0.05 for comparison of LH vs. HH with Student's t-test for unpaired data), impaired initial oxygen consumption (4+/-1* vs. 7+/-1) after cardiac arrest, an increased CVRI (82+/-12* vs. 50+/-8), the formation of myocardial edema (81.0+/-1.3* vs. 77.5+/-1.2), and poor functional recovery (LVSWI 0.2+/-0.1* vs. 1.0+/-0.1; RVSWI 0.1+/-0.1* vs. 0.5+/-0.1). The absence of lactate production in both groups was in accord with the non-ischemic protocol. CONCLUSIONS: CCABCP with a low hematocrit of 20-25% is cardioprotective. In contrast, CCABCP with a high hematocrit of 40-45% jeopardizes the heart despite avoiding ischemic periods, and should be avoided.

Animals↗
PubMed2001

Geometric mismatch between homograft (allograft) and native aortic root: a 14-year clinical experience.

OBJECTIVES: We evaluated the effect of homograft/native aortic root geometric matching and mismatching on valve survival and myocardial remodeling. METHODS: Between January 1, 1987 and March 2000, a total of 292 patients, aged 1.5-78 years (mean, 46.2 years), underwent freehand subcoronary aortic valve (AVR; n=207) and root (ARR; n=85) replacement with matched and mismatched cryopreserved homografts. All patients had pre- and postoperative two-dimensional Doppler echocardiographic studies. Two-hundred and forty-three survivors, excluding children with complete data on sizing, were followed at a total follow-up time of 1269 patient-years. Seventy percent received matched and 30% received mismatched aortic homografts. The homograft valve sizes ranged from 19 to 28 mm. RESULTS: Hospital death for elective first operation was 2.3%, and late death after a mean follow-up of 52 months was 7.9%. The patient survival at 14 years was 92+/-2%. By linear regression analysis, matched homografts were equal to or 1-2 mm less than the native aortic annulus (r(2)=0.73). The valve survival in patients with AVR and ARR was 72+/-4 and 80+/-8% at 14 years, respectively. The freedom from reoperation was 92+/-5, 77+/-4 and 48+/-10% at 14 years for matched, oversized and undersized homografts, respectively (P=0.001). The postoperative cardiac index of patients with 22 and 24 mm homografts was 3.8-4.1 l/m(2), and there was a regression of the left ventricular mass and end-diastolic diameter (P=0.001). CONCLUSIONS: The aortic homograft offers an excellent long-term clinical result. A mismatched homograft is a risk factor for postoperative aortic incompetence, reinfection with pseudoaneurysmal formation and reoperation for the freehand subcoronary implantation technique during the first 7 years of the postoperative period. It is prudent therefore to avoid mismatched homografts and use rather a properly sized stentless xenograft if a root replacement is not indicated.

Adolescent↗
PubMed2001

Primary cardiac tumours: when is surgery necessary?

OBJECTIVE: Primary cardiac tumours are rare. The literature predominantly contains series on myxomas in adults and only a few long-term series that involve the very different primary cardiac tumours in early childhood. As foetal ultrasonography has continued to improve, cardiac tumours are increasingly detected early before significant symptoms develop. It is a challenge for paediatric cardiologists and surgeons to ascertain which patients need surgery and which will benefit from conservative follow-up. METHODS: A retrospective review of a 10-year period revealed 51 tumours in 26 children (median age: 1 month). Analysis was by presentation, location, associated findings, interventions, histological findings, and clinical course. RESULTS: The most common tumours were rhabdomyomas (29), fibromas (nine), teratomas (two), and haemangiomas (two). The tumour location was the right ventricle in 24 and the left ventricle in 22 patients. The symptoms varied between abnormal heart murmur (20), arrhythmia and conduction abnormalities (ten), obstruction of the outflow tract >30 mmHg (nine), severe cyanosis (three) and congestive heart failure (two). Fourteen children with haemodynamic compromises underwent surgery. There was one post-operative death and one heart transplantation after bridging with an assist device. There was no tumour recurrence even when resection was incomplete. Nine of 13 children with rhabdomyomas had spontaneous tumour regression without intervention. CONCLUSIONS: Most of the cardiac tumours in children are benign. Spontaneous regression is possible not only in rhabdomyoma. Surgical intervention is only required for children who develop relevant clinical symptoms. Total resection of the tumour is not the only therapeutic aim; more important is the restoration of the best possible heart function.

Adolescent↗
PubMed2001

Plasmapheresis and cyclophosphamide in the treatment of humoral rejection after heart transplantation.

BACKGROUND: Clinical reports on humoral rejection after heart transplantation showed that these episodes were often more severe than those mediated through T lymphocytes and that the patient's prognosis was significantly worsened. METHODS: To evaluate the impact of plasmapheresis on the course of humoral rejection with hemodynamic compromise (HRHC) episodes, we retrospectively investigated the records of 1,108 heart transplant patients. All patients received triple-drug immunosuppression (cyclosporine a, azathioprine, prednisone) and cytolytic antibodies for induction. Between April 1986 and December 1990, HRHC episodes were treated with cortisone boli and cytolytic antibodies for at least 3 days (Group A). Between January 1991 and April 1999, HRHC episodes were treated with cortisone boli, cytolytic antibodies, and plasmapheresis for at least 3 days (Group B). All patients who survived their first HRHC episode received cyclophosphamide instead of azathioprine as maintenance immunosuppression. RESULTS: Altogether we observed 29 HRHC episodes. In 11 cases, no therapy could be administered or the therapy regimen did not correspond to either Protocol A or B. In the remaining 18 HRHC episodes, 7 episodes in 7 patients were treated without plasmapheresis (Group A), but only 2 patients survived, whereas in 11 HRHC episodes in 6 patients, therapy included plasmapheresis (Group B) and all patients survived (p = 0.002). Four of 6 patients who received cyclophosphamide after their first HRHC episode experienced at least 1 further HRHC episode. CONCLUSIONS: Plasmapheresis seems to improve outcomes in HRHC. However, cyclophosphamide as a maintenance immunosuppressive drug failed to prevent further humoral rejection episodes.

Adolescent↗