1: Mayo Clin
Health Lett 2002 Jan;20(1):1-3
hypertension. High blood pressure in the lungs.
2: Crit Care
Nurse 2000 Dec;20(6):31-40
epoprostenol: a new therapy for primary pulmonary
Sabo J A, Nord C
Medical Center, St Paul, Minn, USA.
3: Crit Care
Nurse 2000 Apr;20(2):22-8; quiz 29-30
pulmonary hypertension in heart transplantation:
meeting the challenge.
Coe P F.
University of South Carolina, Charleston, USA.
The most common
measurements of pulmonary hypertension include
systolic and mean pulmonary artery pressures, PVR,
and transpulmonary gradient. Pulmonary artery pressures
greater than 50 mm Hg, PVR greater than 6 Woods
units, and transpulmonary gradient greater than 15
mm Hg that are unresponsive to optimal vasodilators
are contraindications to orthotopic heart transplantation.
Therapies used to reduce PVR in the cardiac catheterization
laboratory include high-flow oxygen; sublingual
nitroglycerin; and intravenous inotropic agents, vasodilators,
and selective pulmonary vasodilators. Systemic
hypotension may be an undesirable side effect of
vasodilators. Inhaled agents such as nitric oxide and
prostacyclin are specific to the pulmonary vasculature
and reduce PVR without causing systemic hypotension.
All pharmacological therapies used to optimize
pulmonary hemodynamics before transplantation can be
used during transplantation in patients who are at
high risk for acute right ventricular failure
and death after orthotopic heart transplantation
because of elevated pulmonary hemodynamic values.
Use of larger donor hearts for patients with elevated
PVR and referral for heart-lung transplantation are
potential treatment options. A heterotopic heart
transplantation might also be attempted. However, because
of the poor success with heterotopic transplantation,
other options such as treatment with inhaled pulmonary
vasodilators show much more promise and are associated
with long-term survival after transplantation.
Finally, nursing knowledge and implementation of
transplantation protocols are essential for continued
assessment and management of candidates for heart
transplantation who are cared for in the intensive
care or coronary care unit.
4: N Engl J Med
2001 Nov 15;345(20):1465-72
thromboembolic pulmonary hypertension.
Auger WR, Kerr KM, Rubin LJ.
Medicine, University of California, San Diego,
Medical Center, La Jolla 92037-1300, USA.
5: N Engl J Med
2001 Aug 2;345(5):319-24
the gene for bone morphogenetic protein receptor II as a cause of primary
pulmonary hypertension in a large kindred.
Wheeler L, Lane KB, Loyd E, Gaddipati R, Phillips JA
3rd, Loyd JE.
Center for Lung
Research, Department of Medicine, Vanderbilt
University School of Medicine, Nashville, TN, USA. firstname.lastname@example.org
patients with primary pulmonary hypertension are
thought to have sporadic, not inherited, disease.
Because clinical disease develops in only 10
to 20 percent of persons carrying the gene for familial
primary pulmonary hypertension, we hypothesized that
many patients with apparently sporadic primary
pulmonary hypertension may actually have familial
primary pulmonary hypertension. METHODS: In a study
conducted over 20 years, we developed a registry
of 67 families affected by familial primary pulmonary
hypertension. Through patient referrals, extensive
family histories, and correlation of family pedigrees,
we discovered shared ancestry among five subfamilies.
We established the diagnosis of primary pulmonary
hypertension by direct evaluation of patients and
review of autopsy material and medical records. We assessed
some family members for mutations in the gene
encoding bone morphogenetic protein receptor II
(BMPR2), which has recently been found to cause familial
primary pulmonary hypertension. RESULTS: We linked
five separately identified subfamilies that included
394 known members spanning seven generations, which
were traced back to a founding couple in the
mid-1800s. Familial primary pulmonary hypertension
has been diagnosed in 18 family members, 12 of whom
were first thought to have sporadic
disease. The conditions of 7 of the 18 were initially
misdiagnosed as other cardiopulmonary diseases. Six
members affected with familial primary pulmonary
hypertension and 6 of 10 at risk for carriage have
been undergone genotype analysis, and they have the
same mutation in BMPR2, a transversion of thymine
to guanine at position 354 in exon 3. CONCLUSIONS:
Many cases of apparently sporadic primary pulmonary
hypertension may be familial. Failure to detect
familial primary pulmonary hypertension results from
incomplete expression within families, skipped
generations, and incomplete family pedigrees.
The recent discovery of mutations in BMPR2 should
make it possible to identify those with
susceptibility to disease.
6: Crit Care
Clin 2001 Apr;17(2):453-67
pulmonary hypertension: critical care clinics.
Pulmonary Heart Disease, Rush-Presbyterian-St.
Luke's Medical Center, Chicago, Illinois, USA.
hypertension has many causes and therapies. A
meticulous evaluation is critical. Substantial
advances in medical therapy have occurred over the
past decade, and the future treatment of this
syndrome is promising, with many new medications
on the horizon.
7: South Med J
2001 Jun;94(6):635-9 [Texto
hypertension associated with HIV infection.
Shellito J, Welsh D, de Boisblanc BP.
Pulmonary/Critical Care Medicine, Louisiana State
University Health Sciences Center, New Orleans, USA.
hypertension occurs with increased frequency among
patients with human immunodeficiency virus (HIV)
infection. Although the pathogenesis of HIV-associated
pulmonary hypertension remains unknown, it appears
to occur independently of other risk factors
associated with pulmonary vasculopathy, such as
chronic hepatitis C infection and intravenous drug use.
Signs and symptoms are typical of those
immunocompetent patients with primary pulmonary hypertension,
but because many HIV-infected patients are receiving
intensive medical supervision, the diagnosis of
pulmonary hypertension is often made at an earlier
stage. Acute responses to epoprostenol are similar
to those among non-HIV-infected individuals, but the
benefits of long-term, intravenous treatment
with epoprostenol in HIV-infected patients is
unknown. Future investigations should define the
true incidence of pulmonary hypertension and the
long-term effects of epoprostenol on survival among
HIV-infected individuals. Physicians should be alert
to possible pulmonary hypertension in persons
infected with HIV.
8: Am Fam
Physician 2001 May 1;63(9):1789-98 [Texto
treatment of pulmonary hypertension.
Pulmonary and Critical Care Medicine, University of
Kansas Medical Center, Kansas City, MO 66160-7381,
pulmonary hypertension is a rare disease of unknown
etiology, whereas secondary pulmonary hypertension
is a complication of many pulmonary, cardiac and
extrathoracic conditions. Chronic obstructive pulmonary
disease, left ventricular dysfunction and disorders
associated with hypoxemia frequently result
in pulmonary hypertension. Regardless of the etiology,
unrelieved pulmonary hypertension can lead to
right-sided heart failure. Signs and symptoms of
pulmonary hypertension are often subtle and nonspecific.
The diagnosis should be suspected in patients with
increasing dyspnea on exertion and a known cause of
pulmonary hypertension. Two-dimensional echocardiography
with Doppler flow studies is the most useful imaging
modality in patients with suspected pulmonary hypertension.
If pulmonary hypertension is present, further
evaluation may include assessment of oxygenation,
pulmonary function testing, high-resolution computed
tomography of the chest, ventilation-perfusion lung
scanning and cardiac catheterization. Treatment with
a continuous intravenous infusion of prostacyclin
improves exercise capacity, quality of life,
hemodynamics and long-term survival in patients with
primary pulmonary hypertension. Management of
secondary pulmonary hypertension includes correction
of the underlying cause and reversal of hypoxemia.
Lung transplantation remains an option for selected patients
with pulmonary hypertension that does not respond to
9: Hosp Pract
(Off Ed) 2001 Mar 15;36(3):29-32, 37-40 [Texto
approaches to pulmonary hypertension.
School of Medicine, Providence, RI, USA.
evaluation will often reveal secondary--and perhaps
reversible--factors contributing to pulmonary
hypertension. For primary disease, there are now a variety
of treatments, ranging from calcium channel blockers
10: Heart 2001
arterial hypertension: new ideas and perspectives.
Cardiology, University of Bologna, Italy. email@example.com
11: Chest 2001
continuous inhaled nitric oxide for primary pulmonary
Julia-Serda G, Pulido-Duque JM, Gorriz-Gomez E,
Pneumology, Hospital General de Gran Canaria Dr.
Negrin, Las Palmas de Gran Canaria, Spain. firstname.lastname@example.org
We describe a
case of long-term administration of nitric oxide
(NO) in a 32-year-old man who was admitted with
exertional dyspnea and anasarca. A diagnosis
of primary pulmonary hypertension was made. An acute
vasodilator trial with inhaled NO showed a 5%
reduction of the mean pulmonary artery pressure. Long-term
NO inhalation therapy was initiated. Twenty days
later, the dyspnea improved, the anasarca resolved,
and the PaO(2) level increased. After 12 months of
NO therapy, the patient remained stable and no signs
of toxicity or tachyphylaxis were observed. To our
knowledge, this is the first report of 1 year
of continuously inhaled NO in an adult patient with
primary pulmonary hypertension. These findings
suggest that prolonged NO therapy might be an effective
alternative, at a lower cost, to the continuous IV
infusion of epoprostenol.
12: Chest 2000
pulmonary hypertension: analytic review of 131
Mehta NJ, Khan
IA, Mehta RN, Sepkowitz DA.
Medicine, Long Island College Hospital, Brooklyn,
report two new cases of HIV-related pulmonary
hypertension and to review and analyze the existing
reports on the subject. METHOD: Two new cases of HIV-related
pulmonary hypertension are described, and the cases,
case series, and related articles on the subject in
all languages were identified through a comprehensive
MEDLINE search. RESULTS: Among the 131 reviewed
cases, 54% were male, and the age range was 2 to 56
years (mean, 33 years). The interval between the
diagnosis of HIV disease and the diagnosis of pulmonary
hypertension was 33 months. In 82% of cases,
pulmonary hypertension was related solely to HIV infection.
Presenting symptoms were progressive shortness of
breath (85%), pedal edema (30%), nonproductive cough
(19%), fatigue (13%), syncope or near-syncope (12%),
and chest pain (7%). The mean (+/- SD) pulmonary
arterial systolic BP was 67 +/- 18 mm Hg (n = 116),
and diastolic BP was 40+/-11 mm Hg (n = 39). Pulmonary
vascular resistance was 983+/-420 dyne. s. cm(-5) (n
= 29). Chest radiographs demonstrated cardiomegaly
(72%) and pulmonary artery prominence (71%).
Right ventricular hypertrophy was the most common
electrocardiographic finding (67%). Dilatation of
the right heart chambers was the most common echocardiographic
finding (98%). Plexogenic pulmonary arteriopathy was
the most common histopathology (78%). Pulmonary
function tests demonstrated mild restrictive
patterns with variably reduced diffusing capacities.
The responses to vasodilator agents and
antiretroviral therapy was variable. Sixty-six patients
died during a median follow-up period of 8 months.
The median length of time from diagnosis to death
was 6 months. CONCLUSION: HIV infection is an independent
risk factor for the development of pulmonary
hypertension. The appearance of unexplained
cardiopulmonary symptoms in HIV-infected individuals
should suggest pulmonary hypertension.
13: Mayo Clin
Proc 2000 Jun;75(6):625-30
hypertension: diagnostics and therapeutics.
Pulmonary and Critical Care Medicine, Mayo Clinic,
Rochester, Minn 55905, USA.
hypertension (PH) may develop because of a spectrum
of insults to the lungs; in some patients, there
seems to be no cause. Noninvasive tests, such as standard
chest radiography, electrocardiography, and transthoracic
Doppler echocardiography, provide effective
screening if PH is suspected. This synopsis focuses
on these screening studies and the more common clinical
problems, including primary cardiac abnormalities,
obstructive sleep apnea, chronic pulmonary
embolism, pulmonary parenchymal problems, connective
tissue disorders, cirrhosis with portal
hypertension, and use of appetite suppressants, that
should be considered when PH exists. Treatment
options for PH, including intravenous
prostacyclin (epoprostenol), and investigational
agents such as subcutaneous or oral prostacyclin
analogues and oral endothelin receptor antagonists