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Marius M Hoeper

Publications and source records attributed to Marius M Hoeper.

27 records · Page 2Linked to original sources
PubMed2004

Portopulmonary hypertension and hepatopulmonary syndrome.

The clinically and pathophysiologically distinct entities of portopulmonary hypertension and hepatopulmonary syndrome occur in a substantial proportion of patients who have advanced liver disease of different causes. These disorders are notoriously underdiagnosed, but they have a substantial impact on survival and require focused treatment. Abnormal intrapulmonary vascular dilatation, the hallmark of hepatopulmonary syndrome, can cause profound hypoxaemia that can be very difficult to treat. By contrast, portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodelling that eventually leads to right-heart failure. Insights into the pathogeneses of these syndromes have led to novel therapeutic approaches. However, in severely affected patients, effective treatment remains a difficult task. In selected patients, liver transplantation represents the only treatment option, but the decision to do isolated liver transplantation is particularly challenging in patients who have severe pulmonary disease involvement. Data from several centres have contributed to provide criteria that allow improved prediction of which patients may, or may not, benefit from liver transplantation alone.

Hepatopulmonary Syndrome↗
PubMed2003

Cardiovascular effects of systemic nitric oxide synthase inhibition with asymmetrical dimethylarginine in humans.

BACKGROUND: Increased blood concentrations of the endogenous nitric oxide synthase (NOS) inhibitor asymmetrical dimethylarginine (ADMA) have been linked to excess cardiovascular morbidity and mortality and to progression of renal disease. We evaluated systemic cardiovascular effects of ADMA infusion in healthy subjects using invasive techniques, ie, right heart catheter and inulin/para-aminohippurate clearance. METHODS AND RESULTS: Plasma ADMA concentrations encountered in patients with cardiovascular diseases, ie, between 2 and 10 micromol/L, caused a significant (P<0.05) decrease in concentrations of plasma cGMP, the main second messenger of NO. In addition, cardiac output was significantly lower (5.3+/-0.4 versus 5.8+/-0.6 L/min; P<0.05 versus baseline), and systemic vascular resistance was significantly higher (1403+/-123 versus 1221+/-100 dyn x s x cm(-5); P<0.05 versus baseline). The infusion of 0.25 mg ADMA x kg(-1) x min(-1) or 3 microg N(G)-nitro-L-arginine methyl ester x kg(-1) x min(-1), a potent synthetic NOS inhibitor with long action, resulted in a comparable decrease in effective renal plasma flow (from 670+/-40 to 596+/-29 mL x min(-1); P<0.05) and an increase in renovascular resistance (from 79+/-5 to 90+/-7 mm Hg x mL(-1) x min(-1); P<0.05). Moreover, administration of ADMA caused significant sodium retention and blood pressure increase (both P<0.05). The observed effects of ADMA in the systemic circulation were sustained corresponding to a mean plasma half-life of 23.5+/-6.8 minutes, calculated from plasma ADMA decay curves in healthy subjects. CONCLUSIONS: Systemic ADMA infusion is responsible for a short-term, modest decrease in cardiac output with comparable decrease in effective renal plasma flow while increasing systemic vascular resistance and blood pressure in a dose-related manner.

Arginine↗
PubMed2003

Primary pulmonary hypertension may be a heterogeneous disease with a second locus on chromosome 2q31.

OBJECTIVES: The aim of our study was to identify genetic causes of primary pulmonary hypertension (PPH), to estimate the proportion of families with mutations in the BMPR2 (bone morphogenetic protein receptor type 2) gene, and to examine whether genetic heterogeneity might play a role. BACKGROUND: The BMPR2 mutations have been identified in a substantial portion of patients with familial or sporadic PPH. However, the genetic cause of PPH remains unclear in at least 45% of families. METHODS: We investigated 130 members of 10 families with at least 1 PPH patient, recruited without selection for familial disease. Manifest PPH was documented in 21 individuals. An increase in pulmonary artery systolic pressure (PASP) above 40 mm Hg during supine bicycle exercise was found in 46 healthy individuals. Their PASP increased from 21.0 +/- 4.6 mm Hg at rest to 54.0 +/- 9.8 mm Hg during exercise. In 51 relatives, PASP values were normal at rest and during exercise, and 12 members were classified as status unknown. RESULTS: Two families showed a mutation in the BMPR2 gene. Three families with no BMBR2 mutation showed evidence for linkage to a more proximal location on chromosome 2q31 (odds ratio [OR] for linkage 1.1.10(6):1). This locus, designated PPH2, maps in-between the markers D2S335 and D2S2314. We obtained significant support for heterogeneity in PPH with an OR of 2.8.10(11). CONCLUSIONS: We conclude that PPH may be a genetically heterogeneous disorder with at least two-and possibly more-causative genes.

Adolescent↗
PubMed2003

Technical advances of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension.

OBJECTIVE: To minimize the side-effects of circulatory arrest times and profound hypothermia in patients undergoing pulmonary thromboendarterectomy (PTE) for chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Between March 2000 and June 2002, 30 patients (in New York Heart Association (NYHA) class III or IV) were operated for CTEPH using our modified technique. It includes moderate hypothermic (28-32 degrees C), total cardiopulmonary bypass (CPB) and simultaneous selective antegrade cerebral perfusion and occlusion of the bronchial arteries by introducing an occlusive balloon catheter into the descending aorta. The preoperative pulmonary vascular resistance in the cohort was 873+/-248dynes/s/cm(-5). RESULTS: Mean total CPB, cross-clamp times and duration of anterograde cerebral perfusion were 132+/-40, 98+/-21 and 21+/-10min, respectively. Mean core temperature 29.5+/-1.9 degrees C. The duration of postoperative mechanical ventilatory support was 34+/-44h and the mean stay in the ICU was 5+/-9 days. Seven patients had mild to moderate lung reperfusion injury, one transient neurological dysfunction. Three patients (10%) died during their hospital stay, two for multiorgan failure and one for persistent pulmonary hypertension. All patients had a significant pulmonary hemodynamic improvement and all achieved NYHA class I (P<0.01) status 4 weeks after discharge, remaining stable at a median follow-up time of 16 months (range, 1-29 months) postoperatively. CONCLUSIONS: These technical advances improve neurological outcome, control back-bleeding from bronchial arteries and avoid prolonged rewarming phases in patients undergoing PTE.

Chronic Disease↗
PubMed2002

Inhaled iloprost for severe pulmonary hypertension.

BACKGROUND: Uncontrolled studies suggested that aerosolized iloprost, a stable analogue of prostacyclin, causes selective pulmonary vasodilatation and improves hemodynamics and exercise capacity in patients with pulmonary hypertension. METHODS: We compared repeated daily inhalations of 2.5 or 5.0 microg of iloprost (six or nine times per day; median inhaled dose, 30 microg per day) with inhalation of placebo. A total of 203 patients with selected forms of severe pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (New York Heart Association [NYHA] functional class III or IV) were included. The primary end point was met if, after week 12, the NYHA class and distance walked in six minutes were improved by at least one class and at least 10 percent, respectively, in the absence of clinical deterioration according to predefined criteria and death. RESULTS: The combined clinical end point was met by 16.8 percent of the patients receiving iloprost, as compared with 4.9 percent of the patients receiving placebo (P=0.007). There were increases in the distance walked in six minutes of 36.4 m in the iloprost group as a whole (P=0.004) and of 58.8 m in the subgroup of patients with primary pulmonary hypertension. Overall, 4.0 percent of patients in the iloprost group (including one who died) and 13.7 percent of those in the placebo group (including four who died) did not complete the study (P=0.024); the most common reason for withdrawal was clinical deterioration. As compared with base-line values, hemodynamic values were significantly improved at 12 weeks when measured after iloprost inhalation (P<0.001), were largely unchanged when measured before iloprost inhalation, and were significantly worse in the placebo group. Further significant beneficial effects of iloprost treatment included an improvement in the NYHA class (P=0.03), dyspnea (P=0.015), and quality of life (P=0.026). Syncope occurred with similar frequency in the two groups but was more frequently rated as serious in the iloprost group, although this adverse effect was not associated with clinical deterioration. CONCLUSIONS: Inhaled iloprost is an effective therapy for patients with severe pulmonary hypertension.

Administration, Inhalation↗
PubMed2002

Outcome after cardiopulmonary resuscitation in patients with pulmonary arterial hypertension.

Patients with pulmonary arterial hypertension (PAH) often die from right heart failure or sudden cardiac death. Cardiopulmonary resuscitation (CPR) may be instituted in these patients but there are no data in the medical literature about the outcome of CPR in this group of patients. We conducted a retrospective multicenter international study on the frequency and results of CPR in patients with PAH. A total of 3,130 patients with PAH were treated between 1997 and 2000 in 17 referral centers in Europe and in the United States. During this period, 513 patients had circulatory arrest and CPR was attempted in 132 (26%) of these patients. Although 96% of the CPR attempts took place in hospitalized patients (74% in intensive care units or equally equipped facilities) and although there was only minimal delay between collapse and initiation of CPR, resuscitation efforts were primarily unsuccessful in 104 patients (79%). Only eight patients (6%) survived for more than 90 d; these patients had no residual neurologic deficit. Hemodynamics obtained within 3 mo before CPR did not show any significant differences between the survivors and nonsurvivors. Except for one patient, all long-term survivors had identifiable causes of circulatory arrest that were rapidly reversible. Our data indicate that CPR for circulatory arrest in patients with PAH is rarely successful unless the cause of the cardiopulmonary decompensation can be corrected.

Aged↗