Congenital cardiomyopathy and
pulmonary hypertension: Another fatal variant of cytochrome- c oxidase
deficiency.
J Inherit Metab Dis. 2004;27(6):735-9
Venditti CP, Harris MC, Huff D,
Peterside I, Munson D, Weber HS, Rome J, Kaye EM, Shanske S, Sacconi S, Tay S,
Dimauro S, Berry GT.
Division of Human Genetics and Molecular Biology, The Children's Hospital of
Philadelphia, University of Pennsylvania School of Medicine, Philadelphia,
Pennsylvania.
Summary: Biventricular hypertrophy was noted at 24 weeks' gestation in a fetus
with isolated cytochrome- c oxidase (COX) deficiency. Shock, caused by
hypertrophic cardiomyopathy and severe pulmonary hypertension, led to the
patient's death on day 6. His phenotype defines a new lethal variant of COX
deficiency characterized by prenatal-onset cardiopulmonary pathophysiology.
The serotonin receptor and
transporter as potential therapeutic targets for pulmonary hypertension.
Curr Opin Investig Drugs. 2004 Sep;5(9):963-6
Wang HL.
Department of Clinical Pharmacology, China Medical University, Shenyang 110001,
China. hlwang@mail.cmu.edu.cn
Pulmonary hypertension (PHT) results from compromised pulmonary
vasoconstriction and vascular remodeling. Serotonin (5-hydroxytryptamine/5-HT)
is one of the important vasomotor agents, and its importance in the
pathogenesis of PHT is currently being investigated. In most mammalian species,
PHT can result from numerous serotonergic drugs, demonstrating that various
5-HT receptor subtypes and the 5-HT transporter (5-HTT) contribute to PHT. Both
are therefore potential therapeutic targets for the treatment of the disorder.
This review describes current awareness of the roles of 5-HT, the 5-HT receptor
and the 5-HTT in PHT.
End-stage heart failure with
pulmonary hypertension: levosimendan to evaluate for heart transplantation
alone versus combined heart-lung transplantation.
Transplantation. 2004 Oct 27;78(8):1237-8
Schulze-Neick I, Luther YC, Ewert P,
Lehmkuhl HB, Hetzer R, Lange PE.
Publication Types:
·
Case Reports
·
Letter
Pulmonary hypertension in a patient
with rheumatoid arthritis treated with leflunomide.
Rheumatology (Oxford). 2004 Nov;43(11):1451-3
Martinez-Taboada VM,
Rodriguez-Valverde V, Gonzalez-Vilchez F, Armijo JA.
Publication Types:
·
Letter
Adrenomedullin in the treatment of
pulmonary hypertension.
Peptides. 2004 Nov;25(11):2013-8
Nagaya N, Kangawa K.
Department of Internal Medicine, National Cardiovascular Center, 5-7-1
Fujisjirodai, Suita, Osaka 565-8565, Japan. nagayann@hsp.ncvc.go.jp
Adrenomedullin (AM) is a potent, long-lasting pulmonary vasodilator peptide.
Plasma AM level is elevated in patients with primary pulmonary hypertension
(PPH), and circulating AM is partially metabolized in the lungs. These findings
suggest that AM plays an important role in the regulation of pulmonary vascular
tone and vascular remodeling. We have demonstrated the effects of three types
of AM delivery systems: intravenous administration, inhalation, and cell-based
gene transfer. Despite endogenous production of AM, intravenously administered
AM at a pharmacologic level decreased pulmonary vascular resistance in patients
with PPH. Inhalation of AM improved hemodynamics with pulmonary selectivity and
exercise capacity in patients with PPH. Cell-based AM gene transfer ameliorated
pulmonary hypertension rats. These results suggest that additional administration
of AM may be effective in patients with pulmonary hypertension. AM may be a
promising endogenous peptide for the treatment of pulmonary hypertension.
Pulmonary hypertension in sickle
cell disease.
Am J Med. 2004 Nov 1;117(9):665-9
Ataga KI, Sood N, De Gent G, Kelly
E, Henderson AG, Jones S, Strayhorn D, Lail A, Lieff S, Orringer EP.
Department of Medicine, University of North Carolina, Chapel Hill, North
Carolina, USA. kataga@med.unc.edu
BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ
damage in patients with sickle cell disease. We sought to determine the
prevalence of pulmonary hypertension in adult patients with sickle cell disease
and to identify factors associated with this life-threatening complication.
METHODS: Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13
years) followed at a University Medical Center were evaluated. They were selected
by a systematic sampling of patients presenting to the clinic for routine
follow-up visits. All enrolled subjects underwent a clinical examination,
Doppler echocardiography, pulmonary function tests, and hematologic tests
during a single visit. Pulmonary hypertension was defined using an age- and
body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary
hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up
to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had
severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression
model, both lower fetal hemoglobin level and lower systolic blood pressure were
associated with the presence of pulmonary hypertension. CONCLUSION: We found that
the prevalence of pulmonary hypertension in adult patients with sickle cell
disease was substantial, particularly in those with lower levels of fetal
hemoglobin and lower systolic blood pressure.
Outcome of patients after surgical
closure of ventricular septal defect at young age: longitudinal follow-up of
22-34 years.
Eur Heart J. 2004 Jun;25(12):1057-62
Roos-Hesselink JW, Meijboom FJ, Spitaels SE, Van Domburg R, Van Rijen EH, Utens EM, Bogers AJ, Simoons ML.