PubMed Health⌕ Search

Biomedical subjects

John Hess

Publications and source records attributed to John Hess.

At least 19 recordsLinked to original sources
PubMed2006

Presence of a ventricular septal defect and the Mustard operation are risk factors for late mortality after the atrial switch operation: thirty years of follow-up in 417 patients at a single center.

BACKGROUND: Survival and functional status of patients with transposition of the great arteries treated by atrial switch are reported to be reasonably good within the first 15 postoperative years. However, in some patients, the function of the systemic right ventricle deteriorates, leading to significant morbidity or even to late mortality. This study seeks to identify risk factors for late death. METHODS AND RESULTS: Records of 329 patients after the Senning operation and 88 after the Mustard operation at a single center were retrospectively reviewed for demographic, anatomic, and echocardiographic predictors and outcomes. Mean follow-up interval was 19.1+/-6.5 years and was 95% complete. Survival 25 years after the Mustard procedure was 75.9+/-4.8% and after the Senning procedure was 90.9+/-2.3% (P=0.002). Mustard patients died more often of arrhythmia than Senning patients (P<0.001) and needed more baffle-related reoperations (P<0.0001). Ventricular septal defect closure at the time of the atrial switch operation (hazard rate=2.3; 95% confidence interval, 1.1 to 4.7; P=0.025) and the Mustard operation (hazard rate=2.0; 95% confidence interval, 1.01 to 3.8; P=0.045) emerged as independent risk factors for late mortality in multivariate analysis. At follow-up, 85.8% of the patients led a normal life with full-time work, and 11.8% were able to do part-time work. Only 2.4% experienced noticeable limitation of activities. CONCLUSIONS: Our patient data reveal satisfactory results at long term in this historic collective. Patients who had undergone ventricular septal defect closure at the time of the atrial switch operation and those who had undergone a Mustard operation are at higher risk for late death. Close follow-up, especially of these subgroups, is warranted.

Adolescent↗
PubMed2006

Characteristics of Doppler myocardial echocardiography in patients with tricuspid atresia after total cavopulmonary connection with preserved systolic ventricular function.

BACKGROUND: Doppler myocardial echocardiography (DME) may be an excellent additional means of assessing determinants of systolic and diastolic ventricular function in patients with tricuspid atresia (TA) after total cavopulmonary connection (TCPC). METHODS AND RESULTS: Thirty-three patients with TA and normal systolic shortening/ejection fraction (M-mode) after TCPC were studied by DME at a median age of 7.6 years (range 1.5-17 years). These results were compared to 16 age matched normal controls. Median time under a cavopulmonary shunt was 5.2 years (range 0.6-13.3 years). Isovolumic acceleration and isovolumic velocity did not differ significantly. All other systolic (S-wave acceleration-velocity and S-wave duration) and diastolic DME indices (E-deceleration-velocity, A-velocity and E/A ratio) were significantly lower in TCPC patients in comparison to normals (p<0.0001). Furthermore, isovolumic relaxation time (IRT) was significantly prolonged in the patient group (p<0.0001). Even though there was a significant correlation between the time of volume unloading and left ventricular end-diastolic diameter, exclusion of all patients with short period of volume unloading and relatively large systemic ventricles did not alter the results. CONCLUSIONS: Patients with TA after TCPC and normal shortening and ejection fraction have normal isovolumic acceleration. Load dependent DME indices of systolic and diastolic function, however, were significantly reduced. These findings suggest that the described DME indices mirror the "normal" hemodynamics after TCPC in patients with TA. It needs to be assessed, whether this is an index of adverse prognosis in long-term surveillance of these patients.

Adolescent↗
PubMed2006

Acute graft-versus-host disease in pancreas transplantation: a comparison of two case presentations and a review of the literature.

As a complication of solid organ transplantation, acute graft-versus-host disease (GVHD) is most associated with small bowel and liver transplants. We present two cases of acute GVHD following pancreas transplantation. Case 1 was a 27-year-old female who underwent cadaveric pancreas transplant 9 months after a successful live donor kidney transplant. Case 2 was a 38-year-old male who received a simultaneous cadaveric pancreas and live donor kidney transplant. Both patients presented within 30 days of transplant with nonspecific symptoms. Rejection and infection were ruled out. Both subjects had progressive decline in mentation associated with pancytopenia and hyperbilirubinemia. Rash was not present until late in their hospital course. Skin biopsies demonstrated mixed chimerism with pancreas donor DNA diagnostic of GVHD. Acute GVHD is a rare, often fatal, complication of pancreas transplantation, and its presentation appears to differ from acute GVHD associated with stem cell transplantation.

Acute Disease↗
PubMed2006

The serum- and glucocorticoid-inducible kinase Sgk-1 is involved in pulmonary vascular remodeling: role in redox-sensitive regulation of tissue factor by thrombin.

The stress-responsive serum- and glucocorticoid-inducible kinase Sgk-1 is involved in osmoregulation and cell survival and may contribute to fibrosis and hypertension. However, the function of Sgk-1 in vascular remodeling and thrombosis, 2 major determinants of pulmonary hypertension (PH), has not been elucidated. We investigated the role of Sgk-1 in thrombin signaling and tissue factor (TF) expression and activity in pulmonary artery smooth muscle cells (PASMC). Thrombin increased Sgk-1 activity and mRNA and protein expression. H2O2 similarly induced Sgk-1 expression. Antioxidants, dominant-negative Rac, and depletion of the NADPH oxidase subunit p22phox diminished thrombin-induced Sgk-1 expression. Inhibition of p38 mitogen-activated protein kinase, phosphatidylinositol 3-kinase, and phosphoinositide-dependent kinase-1 prevented thrombin-induced Sgk-1 expression. Thrombin or Sgk-1 overexpression enhanced TF expression and procoagulant activity, whereas TF upregulation by thrombin was diminished by kinase-deficient Sgk-1 and was not detectable in fibroblasts from mice deficient in sgk-1 (sgk1(-/-)). Similarly, dexamethasone treatment failed to induce TF expression and activity in lung tissue from sgk1(-/-) mice. Transcriptional induction of TF by Sgk-1 was mediated through nuclear factor kappaB. Finally, Sgk-1 and TF proteins were detected in the media of remodeled pulmonary vessels associated with PH. These data show that thrombin potently induces Sgk-1 involving NADPH oxidases, phosphatidylinositol 3-kinase, p38 mitogen-activated protein kinase, and phosphoinositide-dependent kinase-1, and that activation of nuclear factor kappaB by Sgk-1 mediates TF expression and activity by thrombin. Because enhanced procoagulant activity can promote pulmonary vascular remodeling, and Sgk-1 and TF were present in the media of remodeled pulmonary vessels, this pathway may play a critical role in vascular remodeling in PH.

3-Phosphoinositide-Dependent Protein Kinases↗
PubMed2006

Implantable cardioverter defibrillator (ICD) in children.

BACKGROUND: Implantable cardioverter defibrillators (ICD) proved to be effective in the prevention of sudden cardiac death in adults. In children, the experience of ICD therapy is limited. This retrospective study was undertaken to review our experience with ICD implantation in children with special consideration of psychosocial impact of this therapy. METHODS AND RESULTS: Sixteen children (f:5, m:11, median age 12.2 years, range 4-15.9 years) received an ICD. Eleven patients had survived sudden cardiac death with documented ventricular fibrillation (VF) and five patients had sustained ventricular tachycardia (VT) with hemodynamic significance. The underlying heart disease was congenital in 5, hypertrophic cardiomyopathy in 2, myocarditis in 2 and primary electrical in 7 patients. All leads were implanted transvenously. Mean follow up was 43.1 months (range 1-105 months). All patients are alive. In 7 patients, a total of 387 sustained VT episodes were detected by the ICD. At follow-up, 10 inappropriate shocks were delivered in four patients. One early and six late lead revisions were done in seven patients. 12/16 (75%) patients had concomitant antiarrhythmic drug therapy. About half of the adolescents showed signs of depression and/or anxiety. CONCLUSION: ICD therapy via transvenous access for prevention of sudden cardiac death is feasible and effective even in small children. However, the occurrence of lead complications is significant. Since about half of the adolescents showed signs of depression and/or anxiety, professional psychological surveillance should be considered in these patients.

Adolescent↗
PubMed2006

The fate of systemic blood pressure in patients after effectively stented coarctation.

AIMS: The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults. METHODS AND RESULTS: Forty-three patients (native CoA, 8; female, 12) were treated with stent implantation at a median age of 16.8 years (range 7.9-44.8 years). Only stents dilatable to an adult size aorta were implanted. All but two patients with functionally univentricular hearts had arterial hypertension. Exercise tests, 24-h blood pressure, clinical examination, echocardiography, and elective catheterization were used to assess follow-up. The narrowed segment was widened significantly from a median of 8 to 12.4 mm (P < 0.0005). The peak-to-peak gradient between the ascending and the descending aorta was lowered significantly from a median of 22 mmHg to 1 mmHg (P < 0.0005). No major complications occurred. The systolic blood pressure at the right arm was lowered significantly (P < 0.0005) from 144 mmHg before stent implantation to 128 mmHg at the last visit. At a median follow-up of 30 months (3-72 months), 68% of all patients were classified to be normotensive. CONCLUSION: Stent implantation for selected patients with recurrent and native CoA is safe and may effectively reduce the blood pressure gradient across the CoA site. We suggest using only stents dilatable to an adult size aorta. However, arterial hypertension persists in a significant number of the patients. Impaired elastic properties of the aorta may be the cause for this finding.

Adolescent↗
PubMed2006

Livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity (4G/4G) treated successfully with tissue plasminogen activator.

BACKGROUND: Livedoid vasculopathy (LV) is an occlusive thrombotic disease that affects primarily the small blood vessels of the lower extremities and often is associated with recurrent painful ulcerations. The pathogenesis of LV is unclear, but the disease is largely attributed to a hypercoagulable state. Factor V Leiden mutation, heterozygous protein C deficiency, homozygous hyperhomocysteinemia, and other inherited thrombophilias have been associated with LV. Plasminogen activator inhibitor-1 (PAI-1) is an important inhibitor of the fibrinolytic system. Elevated levels of PAI-1 are found in some patients with thrombotic diseases. Some of these patients are homozygous for an allele of PAI-1 containing a stretch of 4 guanines at base -675 in the promoter region. This variant is associated with elevated PAI-1 protein levels, impaired fibrinolysis, and increased risk of thrombosis. OBSERVATIONS: A 33-year-old white woman had a 3-month history of painful enlarging ulcers on both ankles. Various therapies, including administration of oral antibiotic agents and prednisone up to 100 mg/d, to treat presumed vasculitis, were unsuccessful. Skin biopsy specimens revealed numerous thick-walled small blood vessels, many of which were filled with fibrin thrombi, in association with minimal perivascular inflammatory infiltrate, extensive epidermal necrosis, and focal ulceration. A diagnosis of thrombotic vasculopathy was made. Clinical workup revealed an elevated plasma level of PAI-1 (31 microM/mL; reference range, <25 microM/mL) and PAI-1 promoter 4G/4G homozygosity detected at DNA sequencing. Treatment with heparin sodium and tissue plasminogen activator dramatically improved the lesions, resulting in complete healing of the ulcerations. Continuation of anticoagulant therapy with warfarin sodium and episodic administration of tissue plasminogen activator was required for symptomatic control. CONCLUSIONS: Patients with LV may have elevated plasma PAI-1 levels. This may be associated with the PAI-1 promoter 4G/4G genotype, which has not previously been linked with LV. Further studies in patients with LV are warranted to determine how frequently this genotype is present because it may identify responsiveness to fibrinolytic therapy.

Adult↗
PubMed2006

Impaired cardiac autonomic nervous activity predicts sudden cardiac death in patients with operated and unoperated congenital cardiac disease.

OBJECTIVES: Sudden cardiac death is a leading cause of mortality in patients with congenital cardiac disease after surgical correction and is potentially preventable. The identification of patients at risk is therefore of major interest. We sought to assess the prognostic value of impaired cardiac autonomic nervous activity in patients with congenital cardiac disease. METHODS: Forty-three consecutive patients with congenital cardiac disease were included in this prospective study. Parameters of heart rate turbulence and heart rate variability were calculated from Holter electrocardiograms. In addition, serum brain natriuretic peptide levels were measured. A combined end point of sudden cardiac death or nearly missed sudden cardiac death was used. RESULTS: During a mean follow up of 27 +/- 12.7 months, 5 patients died, and another 2 were successfully resuscitated. On univariate analysis, both brain natriuretic peptide levels and parameters of heart rate variability and heart rate turbulence were associated with impaired prognosis. On multivariate analysis, pathologic heart rate turbulence was found to be the strongest independent risk stratifier (hazard ratio, 61.5; P < .001). CONCLUSIONS: Impaired cardiac autonomic nervous activity is associated with an increased risk of sudden cardiac death in congenital cardiac disease. Our results suggest that heart rate turbulence might be superior to established markers of cardiac autonomic dysfunction, such as heart rate variability. The combined use of heart rate turbulence, heart rate variability, and markers of neurohormonal activation, such as brain natriuretic peptide, might further improve the prognostic value.

Adult↗
PubMed2005

Prevalence and clinical role of GBV-C infection after cardiac surgery in childhood: a study on 414 patients.

OBJECTIVES: GB-virus C (GBV-C) and hepatitis C virus (HCV) share similar modes of transmission. We, therefore, examined the prevalence and clinical role of GBV-C and HCV in patients after cardiac surgery in childhood. METHODS: We analysed blood samples of 414 patients and compared them to 487 controls. Evidence of liver disease and risk factors for infection was analysed. RESULTS: Overall prevalence of GBV-C infection was 22.5% in the patients, compared to 6.2% in the controls (HCV infection 11.3 vs. 0.7%). GBV-C RNA was detected in 8.2% of the patients vs. 3.7% in the controls (HCV RNA in 6 and 0%, respectively). Eleven patients had detectable RNA of GBV-C and HCV. 63.4% of patients infected with GBV-C and 46.8% of patients infected with HCV cleared the virus from circulation. GBV-C infection was not associated with hepatitis. Liver disease was not more frequent in patients co-infected with HCV and GBV-C. CONCLUSIONS: before 1991 have a substantial risk for HCV and GBV-C infection. However, GBV-C infection seems not to be associated with a liver disease. Co-infection with GBV-C and HCV has no influence on long-term clinical outcome or viral clearance of HCV infection.

Adolescent↗
PubMed2005

Impaired elastic properties of the ascending aorta in newborns before and early after successful coarctation repair: proof of a systemic vascular disease of the prestenotic arteries?

BACKGROUND: Despite successful surgical correction, morbidity of patients with coarctation of the aorta is increased. It is well known that these patients have impaired elastic properties of the prestenotic arteries. To find out whether these abnormalities are primarily present or develop later, we studied 17 newborns before and early after surgical repair. METHODS AND RESULTS: Aortic wall stiffness index and distensibility were calculated using ascending and abdominal aortic diameters determined by M-mode echocardiography and noninvasive estimation of aortic pulse pressure in the right arm and leg. Seventeen patients with aortic coarctation (mean age, 20+/-26 days) were compared with 17 normal neonates (mean age, 13+/-7 days) preoperatively and postoperatively (10+/-6 days after surgery). Ascending aortic distensibility in patients was significantly reduced preoperatively (79+/-58 versus 105+/-36; P=0.03) and postoperatively (65+/-24 versus 105+/-36; P<0.005). Preoperative and postoperative ascending aortic stiffness index was higher in patients (preoperative, 5.2+/-4.4 versus 2.7+/-0.9; P=0.04; postoperative, 4.0+/-1.6 versus 2.7+/-0.9; P<0.005). Elastic properties of the descending aorta did not differ preoperatively or postoperatively compared with those in normal subjects. CONCLUSIONS: Elastic properties of the prestenotic aorta of patients with coarctation seem to be impaired primarily, even in neonates, and remain unchanged early after successful operation. Surgical correction does not resolve inborn pathology of the prestenotic aortic vascular bed.

Aorta↗