Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3736_Библиотеки_им_академика_М_И_Перельмана
.pdf
Heart Failure withPreserved
https://t.me/medicina_free
Ejection Fraction (HFpEF)
CarolinaD.Tennyson
21
Anatomy andPhysiology
Table 21.1 Risk factors for HFpEF
221
222
https://t.me/medicina_free
C. D. Tennyson
Table 21.2 Some etiologies of HFpEF
Classication (See Chap. 20)
Imaging (See Chap. 20)
Diagnosis
Physical Exam (See Chap. 20)
Management
Medical Therapy
21 Heart Failure withPreserved Ejection Fraction (HFpEF)
https://t.me/medicina_free
223
Nonmedical Management
Clinical Pearls
224
https://t.me/medicina_free
References
C. D. Tennyson
Pulmonary Vascular Disease
T
pressure=−
PV
=
()
//
https://t.me/medicina_free
LynShelton andJoeMishkin
22
Introduction
Pulmonary hypertension, in its basic denition, is
the elevation of pulmonary artery pressures. The
elevated pulmonary artery pressures result
from pulmonary vasculature remodeling due to
an underlying disease process. Over time the
resultant progressive workload imposed via the
pulmonary vasculature overworks the right ventricle. PAH without intervention may be progressive, leading to right heart failure and death.
Regarding formal diagnostic criteria, however, pulmonary hypertension consists of a mean
pulmonary artery pressure greater than 20mmHg.
Previously, the cutoff was a mean PA pressure
greater than orequal to 25mmHg based upon an
arbitrary number. Evidence suggested that
patients in the borderline range of a mean PA
pressure 21–24 mmHg had suffered worse outcomeswhichprompted the guideline update. The
PG MeanPApressure Mean pulmonarycapillary wedge
RTPG cardiac output COL
Sixth World Symposium on Pulmonary
Hypertension further denes PH in updated terms
of Pre- and Post-capillary pulmonary hypertension. Patients may also have a combination of
both forms of pulmonary hypertension. These
designations require hemodynamic assessment
via right heart catheterization for pulmonary
artery pressures and pulmonary capillary wedge
pressure measurements, respectively.
Precapillary pulmonary hypertension is
dened as a mean pulmonary artery pressure
(mPAP) greater than 20mmHg at rest in addition
to pulmonary capillary wedge pressure (mPCWP)
less than or equal to 15mmHg and a pulmonary
vascular resistance (PVR) greater than or equal to
3 Wood units. PVR is a function of mean pulmonary artery pressure minus mean wedge givingus
transpulmonary gradient. (TPG). The TPG
divided by cardiac output equals PVR.
min
L. Shelton (*)
Atrium Health/SHVI Heart Failure/Transplant Clinic,
Pulmonary Hypertension Clinic,
Charlotte, NC, USA
Asheville Cardiology, Asheville, NC, USA
e-mail: doyle.shelton@atriumhealth.org
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
R. Musialowski, K. Allshouse (eds.), Cardiovascular Manual for the Advanced Practice Provider,
https://doi.org/10.1007/978-3-031-35819-7_22
J. Mishkin
Atrium Health/SHVI Heart Failure/Transplant Clinic,
Pulmonary Hypertension Clinic, Charlotte, NC, USA
Atrium Health/Sanger Heart and Vascular Institute,
Charlotte, NC, USA
e-mail: Joseph.mishkin@atriumhealth.org
225
226
https://t.me/medicina_free
L. Shelton and J. Mishkin
As you can see from the above equations, specic factors can drive pulmonary vascular resistance. The higher the PA pressure versus the
mean wedge pressure, the higher the PVR.PVR
is augmented when there is lower cardiac output.
Try to think of PVR as a factor of the difculty of
moving blood from the right heart through the
lungs to reach our left heart circulation.
Precapillary PH entails elevated pulmonary
pressures in the absence of left-sided heart disease/
volume overload (i.e., therefore excludes WHO
Group II PH). Isolated post-capillary pulmonary
hypertension is dened as mPAP greater than
20mmHg with mPCWP greater than 15mmHg and
a PVR <3 Wood units. This is consistent with higher
lling pressures secondary to left-sided heart disease (WHO Group II or also can include WHO
Group V). There is also combined pre- and postcapillary pulmonary hypertension where mPCWP
is greater than 15mmHg and, PVR is greater than 3
Wood units. Typically, the pulmonary vascular bed
can vasodilate in response to enhanced ow.
However, if we examine PAH histologically, we
nd remodeling of the distal pulmonary vasculature
with the growth of endothelial and smooth muscle
cells as well as inltration of inammatory cells
[1–3]. This is manifested by constriction via vascu-
lar remodeling with brosis and stiffness. In addition, there is in situ thrombosis [4]. Factors in PH
patients that lead to these changes include decreased
nitric oxide (NO) levels andincreased endothelial
levels. NO is an antiproliferative and a vasodilator,
while endothelin is a vasoconstrictor. Prostacyclin
levels are also decreased. Prostacyclin is antiproliferative, inhibits platelet function, and is a vasodilator. The pathophysiology of PAH has led to the
development of medications that affect these pathways andare targets for treatments [5].
Classications
Table 22.1 WHO group classications of pulmonary
hypertension
Drug and toxin-induced
Heritable
Associated with PAH: Examples
include connective tissue
disorders, portal hypertension,
congenital heart disease, HIV
infection, Pulmonary venous
occlusive disease (PVOD)
II.Pulmonary
hypertension due to
left-sided heart
disease
III.Pulmonary
hypertension due to
lung disease and/or
hypoxia
IV.Chronic
thromboembolic
pulmonary
hypertension
(CETPH).
V.Pulmonary
hypertension with
unclear or
multifactorial
mechanisms
LV systolic or diastolic
dysfunction, valvular heart
disease
Examples include COPD, ILD,
OSA
Pulmonary embolism
Examples include end-stage
renal disease on dialysis,
myeloproliferative disorders,
sarcoidosis
of underlying etiologies, placing them in more
than one WHO group. Pharmacological treatment strategies will be outlined in further detail
later but focus on WHO group I PAH as well as a
pharmacological indication for WHO group IV.
TheGlobal prevalence of PAH is often difcult to assess. European registries have reported
rates of 5–52 per million people. RegardingWHO
Group I, statistics note an annual incidence of
2–5 cases per million people and affects 25 persons per one million population in Western countries. Contrasting this with WHO group 2,
valvular left-sided heart disease accounts for
more than 100 million persons [6, 7].
We need to further categorize pulmonary hypertension based upon the underlying disease process. The World Health Organization (WHO)
designates ve classication groups of pulmonary hypertension (Table 22.1). It should be
noted that although patients may have a diagnosis
of PH, often these patients possess a combination
Presentation/Physical Exam
Findings
As an APP, you will be required to evaluate and
treat patients with primary cardiac issues, but
often they possess concomitant comorbidities.
Patients frequently present with complaints of
22 Pulmonary Vascular Disease
https://t.me/medicina_free
Table 22.2 Physical exam ndings that may be suggestive of pulmonary hypertension
An increased pulmonic
component of the second
heart sound [11].
Holosystolic murmur of
tricuspid regurgitation
Liver tenderness or
enlargement on exam
Cyanosis and evidence of
clubbing may be present
inpatients with underlying
shunts or congenital heart
disease
High-pitched early diastolic
murmur of pulmonic
regurgitation
Elevated jugular venous
distention corresponding to right
ventricular uid overload as well
as tricuspid regurgitation
Right heart failure signs may
include peripheral edema or
ascites
Scleroderma patients may have
associated skin changes,
telangiectasias, digital
ulcerations.
Abnormal pulmonary exam may be associated
with underlying pulmonary diseases such as
interstitial lung disease (ILD), COPD/
emphysema. These include velcro-like dry
crackles, wheezing, or severely diminished
airow
The examination may also include a large A wave
in the jugular venous pulse or may also have
prominent V waves in the jugular venous pulse
secondary to tricuspid regurgitation
Patients may have a palpable RV heave given
right ventricular hypertrophy/dilation
227
dyspnea, fatigue and progressive functional limitations. This presentation may be consistent with
pulmonary HTN, but the differential diagnosis is
lengthy. Given that presenting symptoms can
often be attributed to other comorbidities, PH
patients may have a delay in diagnosis and subsequent treatment.
REVEAL (Registry to Evaluate Early and
Long-Term PAH Disease Management) data
review in 2011 noted 21.1% of patients experienced symptoms greater than 2years before PAH
was recognized. Patients less than 36years of age
showed the highest likelihood of delayed disease
recognition as well as those patients with adocumented history of common respiratory diseases
with obstructive sleep apnea/obstructive airways
disease [8]. Therefore, a detailed history and
proper examination combined with appropriate
diagnostic testing are paramount. Higher risk
comorbidities, such as ahistory of connective tissue disorder, liver disease, HIV disease, thromboembolic history, or methamphetamine abuse,
should imply a higher suspicion for pulmonary
arterial hypertension [1]. This should also include
those with ahistory of congenital heart disease.
More advanced PAH may present with chest
pain, syncope,and evidence of right heart failure/strain. Chest pain can be seen due to reduced
cardiac output as a factor of RV strain and overload in combination with higher pulmonary vascular resistance. Chest pain may also be caused
by left main coronary artery compression secondary to an enlarged pulmonary artery [9].
Syncope in patients with underlying pulmonary
arterial hypertension is highly concerning for
poor cardiac output with RV dysfunction,
reduced forward ow, and high pulmonary vascular resistance. Patientswith right heart failure
and strain may present with prominent abdominal distention/ascites as well as lower extremity
edema and JVD (see discussion on cor pulmonale). Rare ndings may include hoarseness via
Ortner’s syndrome, in which the left laryngeal
nerve becomes paralyzed secondary to compression by dilated pulmonary artery [10].
Work-up and subsequent treatment options will
be based on the type of diagnosed pulmonary
hypertension and associated comorbidities. A
detailed physical exam is an essential component of assessing the pulmonary hypertension
patient (Table22.2).
Diagnostic Modalities/Imaging
Diagnostic testing is necessaryto assist in elucidating the form of pulmonary hypertension to
guide your treatment strategy. Testing should aid
in conrming or excluding forms of pulmonary
hypertension, for which the management strategy
should be focused on the underlying disease process versus PAH.Examples include PH second-
228
https://t.me/medicina_free
L. Shelton and J. Mishkin
ary to obstructive sleep apnea, chronic pulmonary
disease, systolic and diastolic heart failure.
Echocardiography
Transthoracic echo (TTE) is one of the hallmarks
of pulmonary hypertension screening tests. One
benet isthat's it's noninvasive and widely available. It can be a useful initial screening study in
the setting of presenting subjective symptoms.
TTE is effective at identifying structural changes
that may be associated with pulmonary hypertension. These include right ventricular size and systolic function, presence of pericardial effusion,
andpresence and severity of tricuspid regurgitation. It can assess for attening of the interventricular septum (D-shaped LV) associated with
right ventricular pressure and/or volume overload
(Fig.22.1). In addition, an echocardiogram can
identify other potential contributing factors to
pulmonary hypertension including diastolic dysfunction, valvular heart disease, and left ventricular systolic dysfunction.
TTE has been used to estimate pulmonary
artery systolic pressure (PASP) or right ventricular systolic pressure (RVSP) at times. It is not
recommended to useestimated pulmonary artery
pressure, however, via echo for diagnosis. This
given potential inaccuracies of estimated right
atrial pressure as well as suboptimal tricuspid
regurgitation signal or interpretation, which are
used to estimate PA pressures [12]. Furthermore,
TTE ndings should never be utilized in the place
of right heart catheterization for documenting
denitive pulmonary artery systolic pressure for
initiation or alteration of therapies for PAH.
However, tricuspid regurgitation velocity
(TRV) has been utilized for assigning the echocardiographic probability of pulmonary hypertension in patients suspected of havingpulmonary
hypertension. TRV greater than 3.4 m/sec confers a high risk of pulmonary hypertension,
whereas below 2.8m/sec without other signs of
pulmonary hypertension changes on echo confers a low probability if no other parameters of
PH ndings on echo are met [13]. A TRV of less
than 2.8m/sec without other presence of pulmonary hypertension signs on echo confers a low
probability.
Fig. 22.1 D-shaped interventricular septum of PH and
RV enlargement. The RV is severely dilated and larger
than the LV.High RV pressures atten the septum into a
D-shape
Computerized Tomographic
Angiography (CTA) oftheChest
CTA is used to assess for acute pulmonary embolism given concern for thromboembolic phenomenon as acute potential cause for pulmonary
hypertension and right ventricular systolic dysfunction. It should be noted that CTA is an appropriate modality for theevaluation of underlying
acute pulmonary emboli. However, its sensitivity
may be suboptimal for dening chronic thromboembolic phenomenon in WHO IV. (Please see
V/Q scan discussion below).
CT oftheChest
Obtained for parenchymal lung disease, assess
for RV dilation, assess enlarged main pulmonary
artery.
22 Pulmonary Vascular Disease
https://t.me/medicina_free
229
Ventilation Perfusion Scan
This scan canidentify potential chronic thromboembolic phenomenon as CTA chest has diminished sensitivity in identifying chronic
thromboembolic pulmonary disease. Even if a
patient has a negative CTA chest for pulmonary
embolism, this does not exclude thepotential for
chronic thromboembolic disease.
V-Q scan utilizes an inhaled radiolabeled
aerosol and injectable radioactive tracer to assess
lung ventilation/perfusion. A nuclear camera is
utilized to register distribution of the radioactive
material on the alveoli and pulmonary arteries,
looking for mismatches. Results are noted as
high, intermediate, or low probability and nondiagnostic [14].
PFTs: Pulmonary Function Studies
This functional test helps identify pulmonary
hypertension attributed to WHO group III with
the suggestion of underlying restrictive or
obstructive lung disease. PFTs also utilize DLCO
(diffusion capacity), which can be noted to be
decreased in pulmonary arterial hypertension and
concern for PVOD (pulmonary venous occlusive
disease.)
PAH therapy and subsequent titrations based on
follow-up hemodynamics after therapy is initiated. (See Chap. 2).
Chest X-ray
May show enlargement of the pulmonary arteries/RV enlargement.
12 Lead ECG
EKG Findings in pulmonary hypertension may
include right axis deviation, p pulmonale c/w
right atrial enlargement, signs of RV hypertrophy, RV strain, RBBB, and in some cases QTc
prolongation [15].
OSA Evaluation
Sleep apnea may be a contributing factor to WHO
Group III pulmonary hypertension which, can be
readily diagnosed and treated. We will discuss
sleep apnea in further detail later.
Vasoreactivity Study
Cardiac MRI
CMRI is the gold standard for right ventricular
assessment as itcan give accurate measurements
of anatomy, ejection fraction, ow, and even
assess for myocardial perfusion.
Right Heart Catheterization
This invasive study is required for the diagnosis
of pulmonary arterial hypertension as it provides
direct hemodynamic assessment. It is mandatory
to conrm the presence of and help delineate
the type of pulmonary hypertension (pre/post/
combined), and assist with risk stratication. It
also provides the hemodynamic data to initiate
This study isdone at the time of theinitial right
heart cath. This involves assessing pulmonary
pressure changes with a Vaso-reactive agent: typically, this is inhaled nitric oxide. Vaso-reactive
patients demonstrate a reduction in mean pulmonary artery pressure of ≥10mmHg to an absolute
value of ≤40mmHg with either an increase or no
change in cardiac output (CO). Treatment of Vaso
reactive patients will be discussed under treatment options.
6-min Walk
This easy evaluation is the measurement of distance walked in 6 min but is a vital data point
linked to survival rates. This must bemeasured in
a consistently.
230
https://t.me/medicina_free
L. Shelton and J. Mishkin
Pulmonary Artery (PA) Angiogram
A PA angiogram is acatheterization-based procedure to assess for pulmonary emboli. It is typically indicated if abnormal VQ Scan or high
suspicion for chronic thromboembolic disease
(WHO Group IV PH).
Initial Routine Lab Work
1. Complete blood count. Rule out anemia or
potential for possible blood dyscrasias.
2. Complete metabolic panel for assessment of
renal function, liver function studies (portopulmonary HTN).
3. Hepatitis panel.
4. Thyroid panel.
5. HIV serologic testing.
6. Genetic testing: BMPR2. BMPR2 mutation
accounts for 80% of heritable and 20% of
idiopathic pulmonary hypertension [16].
7. Assessment for connective tissue disorders.
For example: ANA.
8. Cardiac BNP or NTProBNP.
Treatment
In terms of pulmonary hypertension management, it is important to verify the type/types of
pulmonary hypertension and risk stratify patients.
Tools are readily available to clinicians to risk
stratify pulmonary hypertension patients into
low, intermediate, and high-risk groups. These
classications are based on functional, clinical,
and hemodynamic measurements. There are
many comprehensive risk stratication tools
available. The following parameters appear to
have the greatest predictive accuracy: 6-min walk
distance, BNP/NTproBNP, right atrial pressure,
cardiac index, andmixed venous oxygen saturation [17].
The REVEAL registry uses variables to calculate 1-year mortality and ispredictive of survival
at baseline, 1-year follow-up, and 5-year follow up. In theabsence of or in conjunction with PAH
pharmacologic therapy when indicated, support-
ive treatments for PAH are an essential component of the treatment paradigm. Basic supportive
treatments should be indicated in the treatment of
an underlying disease process (Table22.3).
Patients with conrmed WHO Group 4 PH
secondary to thromboembolic disease should be
referred early to a specialty center for pulmonary
endarterectomy. If they are not candidates for
surgery, balloon pulmonary angioplasty (BPA)
and medical therapy should be considered.
Table 22.3 Supportive treatment options
Treatment Recommendations
Supervised
exercise
Supplemental
oxygen
Anticoagulation As indicated in WHO Group IV
Diuretics Cautious use for right heart failure
Arrhythmias Aggressive treatment of SVTs.
Avoidance of
pregnancy
Underlying
pulmonary
disease
Smoking
cessation
Immunizations Including PNA/pneumococcal
Psychosocial support
Hematology Correction of iron deciencies
WHO Group IV
(CTEPH)
Avoid over-strenuous exertion/
symptomatic exercise
When required to maintain
appropriate oxygen sats with rest,
exercise, or sleep
(CTEPH)
In idiopathic PAH-must be
determined on an individual basis
as itcan cause reduced right heart
preload
Diuretics for patients with high
left-sided lling pressures (WHO
Group II)
Often poorly tolerated in severe
PAH
Typically try to avoid negative
inotropic medications with right
heart failure
High mortality risk in PAH and
pregnancy. Necessity for
appropriate contraception
Needs appropriate treatment of
underlying pulmonary disease
vaccinations
given increased metabolic demand
with anemia/iron deciency
Early referral to aspecial center for
potential pulmonary
thromboendarterectomy or balloon
pulmonary angioplasty (BPA) if a
candidate
Соседние файлы в папке Библиотека им академика М.И. Перельмана