Hipertensión pulmonar

2022 ESC/ERS GUIDELINES FOR THE

DIAGNOSIS AND TREATMENT OF PH
 Definición hemodinámica

mPAP > 20mmHG en supino y en reposo medido por

cateterismo cardiaco derecho (CCD)
Definición Hemodinámica Grupos clínicos
HP precapilar mPAP > 20 mmHg 1, 3, 4 y 5
PAWP ≤ 15 mmHg
RVP > 2 WU
HP postcapilar mPAP > 20 mmHg 2y5
PAWP > 15 mmHg
RVP ≤ 2 WU
HP combinada mPAP > 20 mmHg 2y5
PAWP > 15 mmHg
RVP > 2 WU
HP de ejercicio mPAP/GC entre reposo y Mal pronóstico
ejercicio > 3 mmHg/min
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
Clínica
 Clínica

Clase Descripción
OMS-FC I Pacientes con HP pero sin consiguiente limitación de la
actividad física. La actividad física ordinaria no causa disnea o
fatiga indebida, dolor torácico o casi síncope
OMS-FC II Pacientes con HP que produzca una ligera limitación de la
actividad física. Están cómodos en reposo. La actividad física
ordinaria causa disnea o fatiga indebida, dolor torácico o casi
síncope
OMS-FC III Pacientes con HP que resulta en una marcada limitación de la
actividad física. Están cómodos en reposo. La actividad
inferior a la habitual provoca disnea o fatiga indebida, dolor
torácico o casi síncope
OMS-FC IV Pacientes con HP con incapacidad para realizar cualquier
actividad física sin síntomas. Estos pacientes manifiestan
signos de IC derecha. La disnea y/o la fatiga pueden
presentarse incluso en reposo. La incomodidad aumenta con
cualquier actividad física.
Diagnóstico

Signos de
enfermedad del
Signos de HP y Signos de
corazón
anormalidades enfermedad
izquierdo/
concomitantes pulmonar
congestión
pulmonar
Agrandamiento Opacificación del Aplanamiento del
del corazón espacio aéreo diafragma
derecho central (EPOC/enfisema)

Agrandamiento Engrosamiento Hiperlucidez

de AP (incluyendo septal (EPOC/enfisema)
dilatación interlobulillar
aneurismática) líneas 'Kerley B'

Vasos Efusiones Pérdida de

periféricos en pleurales volumen
árbol de podar pulmonar
(enfermedad
pulmonar
fibrótica)

Forma de 'botella Agrandamiento Opacificación

de agua' de silueta de la aurícula reticular
cardíaca izquierda (enfermedad
(incluida la carina pulmonar
abierta) fibrótica)
Dilatación del
ventrículo
izquierdo
 Diagnóstico

A: The ventricles B: Pulmonary artery C: Inferior vena cava and RA

RV/LV basal diameter/area RVOT AT <105 ms and/or mid- IVC diameter >21 mm with
ratio >1.0 systolic notching decreased inspiratory collapse
(<50% with a sniff or <20%
with quiet inspiration)
Flattening of the Early diastolic pulmonary RA area (end-systole)
2
interventricular septum (LVEI regurgitation velocity >2.2 m/s >18 cm
>1.1 in systole and/or
diastole)
TAPSE/sPAP ratio PA diameter >AR diameter
<0.55 mm/mmHg PA diameter >25 mm
 Diagnóstico

Recommendations Class Level

Right heart catheterization
It is recommended that RHC is performed to
confirm the diagnosis of PH (especially PAH or I B
CTEPH) and to support treatment decisions
In patients with suspected or known PH, it is
recommended that RHC is performed in I C
experienced centres
*It is recommended that RHC comprises a
complete set of haemodynamics and is I C
performed following standardized protocols
A: The ventricles B: Pulmonary artery C: Inferior vena cava and RA

RV/LV basal diameter/area RVOT AT <105 ms and/or mid- IVC diameter >21 mm with
ratio >1.0 systolic notching decreased inspiratory collapse
(<50% with a sniff or <20%
with quiet inspiration)
Flattening of the Early diastolic pulmonary RA area (end-systole)
2
interventricular septum (LVEI regurgitation velocity >2.2 m/s >18 cm
>1.1 in systole and/or
diastole)
TAPSE/sPAP ratio PA diameter >AR diameter
<0.55 mm/mmHg PA diameter >25 mm
Diagnóstico
 Group 4 (PH associated
Characteristic Group 2 (PH associated Group 3 (PH associated
Diagnostic tool Group 1 (PAH) with pulmonary artery
findings/features with left heart disease) with lung disease)
obstructions)
Clinical presentation Clinical features
Recomendaciones DX
Variable age, but young,
female patients may be
predominantly affected.
Mostly elderly patients,
female predominance in case
of HFpEF.
Mostly elderly patients, male
predominance.
History and clinical findings
Variable age, but elderly male
and female equally affected.
History of VTE (CTEPH may
Clinical presentation depends History and clinical findings suggestive of lung disease. occur in the absence of a VTE
on associated conditions and suggestive of LHD Smoking history common history).
phenotype Risk factors for CTEPH

Oxygen requirement for Uncommon, except for Uncommon Common, often profound Uncommon; common in
hypoxaemia conditions with low DLCO or hypoxaemia in severe PH severe cases with
right-to-left shunting predominantly distal
pulmonary artery occlusions
Chest radiography RA/RV/PA size ↑ LA/LV size ↑ Signs of parenchymal lung RA/RV/PA size ↑
Pruning of peripheral vessels Cardiomegaly disease Number and size of
Occasional signs of peripheral vessels ↓
congestion (interstitial Occasional signs of
oedema/Kerley lines, alveolar pulmonary infarction
oedema, pleural effusion)
Pulmonary function Spirometry/PFT impairment Normal or mildly impaired Normal or mildly impaired Abnormal as determined by Normal or mildly impaired
tests and ABG the underlying lung disease
DLCO Normal or mild-to- Normal or mild-to- Often very low (<45% Normal or mild-to-
moderately reduced (low moderately reduced, predicted) moderately reduced
DLCO in SSc-PAH, PVOD, especially in HFpEF
and some IPAH phenotypes)
Arterial blood gas Normal or reduced Normal or reduced Reduced Normal or reduced
PaO2 Reduced Usually normal Reduced, normal, or Normal or reduced
PaCO2 increased
Echocardiography Signs of PH (increased sPAP, Signs of LHD (HFrEF, Signs of PH (increased sPAP, Signs of PH (increased sPAP,
enlarged RA/RV) HFpEF, valvular) and PH enlarged RA/RV) enlarged RA/RV)
Congenital heart defects may (increased sPAP, enlarged
be present RA/RV)
Lung scintigraphy Planar – SPECT V/Q Normal or matched Normal or matched Normal or matched Mismatched perfusion defect

Chest CT Signs of PH or PVOD Signs of LHD Signs of parenchymal lung Intravascular filling defects,
Pulmonary oedema disease mosaic perfusion, enlarged
Signs of PH Signs of PH bronchial arteries
Signs of PH
Cardiopulmonary High VE/VCO2 slope Mildly elevated Mildly elevated High VE/VCO2 slope
exercise testing Low PETCO2, decreasing VE/VCO2 slope VE/VCO2 slope Low PETCO2, decreasing
during exercise Normal PETCO2, increasing Normal PETCO2, increasing during exercise
No EOV during exercise during exercise No EOV
EOV
Right heart Pre-capillary PH Post-capillary PH Pre-capillary PH Pre- (or post-) capillary PH
catheterization
 Recomendaciones estrategia diagnóstica
Recommendation Class Level
Echocardiography
Echocardiography is recommended as the first-line, non-invasive, diagnostic investigation in
I B
suspected PH
*It is recommended to assign an echocardiographic probability of PH, based on an abnormal TRV
I B
and the presence of other echocardiographic signs suggestive of PH
*It is recommended to maintain the current threshold for TRV (>2.8 m/s) for echocardiographic
I C
probability of PH according to the updated haemodynamic definition
*Based on the probability of PH by echocardiography, further testing should be considered in the
IIa B
clinical context (i.e. symptoms and risk factors or associated conditions for PAH/CTEPH)
*In symptomatic patients with intermediate echocardiographic probability of PH, CPET may be
. IIb C
considered to further determine the likelihood of PH
Imaging
Ventilation/perfusion or perfusion lung scan is recommended in patients with unexplained PH to
I C
assess for CTEPH
CT pulmonary angiography is recommended in the work-up of patients with suspected CTEPH I C
Routine biochemistry, haematology, immunology, HIV testing, and thyroid function tests are
I C
recommended in all patients with PAH, to identify associated conditions
Abdominal ultrasound is recommended for the screening of portal hypertension I C
Chest CT should be considered in all patients with PH IIa C
Digital subtraction angiography should be considered in the work-up of patients with CTEPH IIa C
Other diagnostic tests
Pulmonary function tests with DLCO are recommended in the initial evaluation of patients with PH I C
Open or thoracoscopic lung biopsy is not recommended in patients with PAH III C
 Screening
 Asintomáticos de alto riesgo:  Sintomáticos en grupos de riesgo:
 Esclerosis sistémica  Hipertensión portal
 Portadores de la mutación BMPR2  Infección por VIH
 Familiares de 1º grado de pacientes con HPAH  Enfermedades de tejido conjuntivo (no SS)
 Pacientes en evaluación para trasplante hepático

Systemic sclerosis
*In patients with SSc, an annual evaluation of the risk of having PAH is recommended I B
**In adult patients with SSc with >3 years disease duration, an FVC ≥40%, and a DLCO <60%, the DETECT algorithm is recommended I B
to identify asymptomatic patients with PAH
*In patients with SSc, where breathlessness remains unexplained following non-invasive assessment, RHC is recommended to exclude I C
PAH
*Assessing the risk of having PAH based on an evaluation of breathlessness, in combination with echocardiogram or PFTs and BNP/NT- IIa B
proBNP, should be considered in patients with SSc
*Policies to evaluate the risk of having PAH should be considered in hospitals managing patients with SSc IIa C
**In symptomatic patients with SSc, exercise ETT or CPET, or CMR may be considered to aid decisions to perform RHC IIb C
*In patients with CTD with overlap features of SSc, an annual evaluation of the risk of PAH may be considered IIb C
CTEPH/CTEPD
**In patients with persistent or new-onset dyspnoea or exercise limitation following PE, further diagnostic evaluation to assess for I C
CTEPH/CTEPD is recommended
*For symptomatic patients with mismatched perfusion lung defects beyond 3 months of anticoagulation for acute PE, referral to a I C
PH/CTEPH centre is recommended after considering the results of echocardiography, BNP/NT-proBNP, and/or CPET
Other
*Counselling regarding the risk of PAH and annual screening are recommended in individuals who test positive for PAH-causing I B
mutations and in first-degree relatives of patients with HPAH
*In patients referred for liver transplantation, echocardiography is recommended as a screening test for PH I C
*Further tests (echocardiography, BNP/NT-proBNP, PFTs, and/or CPET) should be considered in symptomatic patients with CTD, IIa B
portal hypertension, or HIV to screen for PAH
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
Grupo I: HAP

PAPm >20mmHg en reposo con PCP

≤15mmHG y RVP ≥3UM en ausencia de

otras causas de HP precapilar.

 Idiopático
 No respondedores a las pruebas de
vasorreactividad
 Respondedores agudos en las pruebas de
vasorreactividad
 Hereditario
 Asociado con drogas y toxinas
 Asociado con:
 Enfermedad del tejido conjuntivo
 Infección por VIH
 Hipertensión portal
 Cardiopatías congénitas
 Esquistosomiasis
 PAH con características de afectación
venosa/capilar (PVOD/PCH)
 PH persistente del recién nacido
 Estratificación de severidad/riesgo
Recommendations Class Level
It is recommended to evaluate disease severity in patients with PAH with a panel of
data derived from clinical assessment, exercise tests, biochemical markers,
echocardiography, and haemodynamic evaluations I B

Achieving and maintaining a low-risk profile on optimized medical therapy is

recommended as a treatment goal in patients with PAH I B

*For risk stratification at the time of diagnosis, the use of a three-strata model (low,
intermediate, and high risk) is recommended, taking into account all available data, I B
including haemodynamics
*For risk stratification during follow-up, the use of a four-strata model (low,
intermediate–low, intermediate–high, and high risk) based on WHO-FC, 6MWD,
and BNP/NT-proBNP is recommended, with additional variables taken into I B
account as necessary

**In some PAH aetiologies and patients with comorbidities, optimization of therapy
should be considered on an individual basis, while acknowledging that a low-risk IIa B
profile is not always achievable
Determinants of prognosis
Low risk Intermediate risk High risk

Pronósico
(estimated 1-year
(<5%) (5–20%) (>20%)
mortality)
Clinical observations and modifiable variables
Signs of right HF Absent Absent Present
Progression of symptoms and No Slow Rapid
clinical manifestations

Syncope No Occasional syncope Repeated syncope

WHO-FC I, II III IV
6MWD >440 m 165–440 m <165 m
CPET Peak VO2 >15 mL/min/kg Peak VO2 11–15 mL/min/kg Peak VO2 <11 mL/min/kg
(>65% pred.) (35–65% pred.) (<35% pred.)
VE/VCO2 slope <36 VE/VCO2 slope 36–44 VE/VCO2 slope >44

Biomarkers: BNP or NT- BNP <50 ng/L BNP 50–800 ng/L BNP >800 ng/L
proBNP NT-proBNP <300 ng/L NT-proBNP 300–1100 ng/L NT-proBNP >1100 ng/L

2 2 2
Echocardiography RA area <18 cm RA area 18–26 cm RA area >26 cm
TAPSE/sPAP TAPSE/sPAP 0.19– TAPSE/sPAP <0.19 mm/mmHg
>0.32 mm/mmHg 0.32 mm/mmHg Moderate or large pericardial
No pericardial effusion Minimal pericardial effusion effusion

cMRI RVEF >54% RVEF 37–54% RVEF <37%

2 2 2
SVI >40 mL/m SVI 26–40 mL/m SVI <26 mL/m
2 2
RVESVI <42 mL/m RVESVI RVESVI >54 mL/m
2
42–54 mL/m
Haemodynamics RAP <8 mmHg RAP 8–14 mmHg RAP >14 mmHg
2 2 2
CI ≥2.5 L/min/m CI 2.0–2.4 L/min/m CI <2.0 L/min/m
2 2 2
SVI >38 mL/m SVI 31–38 mL/m SVI <31 mL/m
SvO2 >65% SvO2 60–65% SvO2 <60%
 Pronóstico
Recommendations Class Level
It is recommended to evaluate disease severity in patients with PAH with a panel of
data derived from clinical assessment, exercise tests, biochemical markers,
echocardiography, and haemodynamic evaluations I B

Achieving and maintaining a low-risk profile on optimized medical therapy is

recommended as a treatment goal in patients with PAH I B

*For risk stratification at the time of diagnosis, the use of a three-strata model (low,
intermediate, and high risk) is recommended, taking into account all available data, I B
including haemodynamics
*For risk stratification during follow-up, the use of a four-strata model (low,
intermediate–low, intermediate–high, and high risk) based on WHO-FC, 6MWD,
and BNP/NT-proBNP is recommended, with additional variables taken into I B
account as necessary

*In some PAH aetiologies and patients with comorbidities, optimization of therapy
should be considered on an individual basis, while acknowledging that a low-risk IIa B
profile is not always achievable
Seguimiento
 Medidas generales
Recommendations Class Level
General measures
**Supervised exercise training is recommended in patients with PAH under medical therapy I A
Psychosocial support is recommended in patients with PAH I C
**Immunization of patients with PAH against SARS-CoV-2, influenza, and Streptococcus
pneumoniae is recommended I C

Diuretic treatment is recommended in patients with PAH with signs of RV failure and fluid
I C
retention
Long-term oxygen therapy is recommended in patients with PAH whose arterial blood oxygen
pressure is <8 kPa (60 mmHg) I C

**In the presence of iron-deficiency anaemia, correction of iron status is recommended in

patients with PAH I C

*In the absence of anaemia, iron repletion may be considered in patients with PAH with iron
IIb C
deficiency
**Anticoagulation is not generally recommended in patients with PAH but may be considered on
an individual basis IIb C

**The use of ACEis, ARBs, ARNIs, SGLT-2is, beta-blockers, or ivabradine is not recommended in
patients with PAH unless required by comorbidities (i.e. high blood pressure, coronary artery III C
disease, left HF, or arrhythmias)
Vasoreactivity testing
Vasoreactivity testing is recommended in patients with I/H/DPAH to detect those who can be treated with high doses of a

Test de vasoreactividad
I B
CCB
It is recommended that vasoreactivity testing is performed at PH centres I C
It is recommended to consider a positive response to vasoreactivity testing by a reduction in mPAP ≥10 mmHg to reach
I C
an absolute value of mPAP ≤40 mmHg with an increased or unchanged CO
*Inhaled nitric oxide, inhaled iloprost, or i.v. epoprostenol are recommended for performing vasoreactivity testing I C
Vasoreactivity testing, for identifying candidates for CCB therapy, is not recommended in patients with PAH other than
III C
I/H/DPAH, and in PH groups 2, 3, 4, and 5
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
Grupo II: HP asociado a cardiopatía izquierda
mPAP> 20 mmHg en reposo y un PAWP
>15 mmHg.
- IpcPH: PVR ≤2 WU
- CpcPH: PVR >2 WU

Prevalencia:
 IC FEVIr → 40-72%
 IC FECIp → 36-83%
 20-30& CpcPH
 Estenosis aór ca → ≈50-60%
 Estenosis mitral severa → ≈ 60-70%
 Diagnóstico
Claves diagnósticas: Rasgo
HP-CI poco
probable
Probabilidad
intermedia
HP-CI
probable
Edad <60 años 60–70 años >70 años
1. Diagnóstico y control de FRCV :Obesidad, Sin factores 1–2 factores >2 factores

la cardiopatía izquierda
HTA, DLP
IGB/DM

de base Presencia de
LHD conocido
No Sí Sí

Intervención No No Sí
2. Evaluación de la HP y cardiaca previa

fenotipado del paciente Fibrilación

auricular
No Paroxística Permanente/pers
istente
LHD estructural No No Presente
3. Diagnóstico ECG Normal o signos HVI leve BRI o HVI

hemodinámico invasivo de distensión del

VD
Ecocardiografía Sin dilatación AI Sin dilatación AI Dilatación AI
 Sospecha PAH o CTEPH E/e′ <13 Flujo mitral <G2 (IVAI>34
2
mL/m )
 Sospecha de CpcHP con HVI
Flujo mitral >G2
componente precapilar CPET Pendiente VE/VC VE/VCO2 elevad ligeramente eleva

grave O 2 altamente
elevado
a
VOE
da VE/VCO 2
VOE
Sin EOV
 IC avanzada y evaluación RMc Sin anomalías en HVI
de trasplante de corazón. el corazón
izquierdo
Dilatación LA
(strain o LA/RA
>1)
 Manejo
Recommendations Class Level
In patients with LHD, optimizing treatment of the underlying condition is recommended
before considering assessment of suspected PH I A

*RHC is recommended for suspected PH in patients with LHD, if it aids management

I C
decisions
*RHC is recommended in patients with severe tricuspid regurgitation with or without LHD
I C
prior to surgical or interventional valve repair
**For patients with LHD and suspected PH with features of a severe pre-capillary
component and/or markers of RV dysfunction, referral to a PH centre for a complete I C
diagnostic work-up is recommended
*In patients with LHD and CpcPH with a severe pre-capillary component (e.g. PVR >5 WU),
I C
an individualized approach to treatment is recommended
*When patients with PH and multiple risk factors for LHD, who have a normal PAWP at rest
but an abnormal response to exercise or fluid challenge, are treated with PAH drugs, close I C
monitoring is recommended
*In patients with PH at RHC, a borderline PAWP (13–15 mmHg) and features of HFpEF,
additional testing with exercise or fluid challenge may be considered to uncover post- IIb C
capillary PH
Drugs approved for PAH are not recommended in PH-LHD III A
No recommendation can be given for or against the use of PDE5is in patients with HFpEF
and combined post- and pre-capillary PH
The use of PDE5is in patients with HFpEF and isolated post-capillary PH is not
III C
recommended
 Manejo
Recommendations Class Level
In patients with LHD, optimizing treatment of the underlying condition is recommended
before considering assessment of suspected PH I A

*RHC is recommended for suspected PH in patients with LHD, if it aids management

I C
decisions
*RHC is recommended in patients with severe tricuspid regurgitation with or without LHD
I C
prior to surgical or interventional valve repair
Tratamiento de la enfermedad de base
**For patients with LHD and suspected PH with features of a severe pre-capillary
component and/or markers of RV dysfunction, referral to a PH centre for a complete I C
diagnostic work-up is recommended ↓
Diuréticos
*In patients with LHD and CpcPH with a severe pre-capillary component (e.g. PVR >5 WU),
an individualized approach to treatment is recommended
I C
*When patients with PH and multiple risk factors for LHD, who have a normal PAWP at rest
but an abnormal response to exercise or fluid challenge, are treated with PAH drugs, close I C
monitoring is recommended
*In patients with PH at RHC, a borderline PAWP (13–15 mmHg) and features of HFpEF,
additional testing with exercise or fluid challenge may be considered to uncover post- IIb C
capillary PH
Drugs approved for PAH are not recommended in PH-LHD III A
No recommendation can be given for or against the use of PDE5is in patients with HFpEF
and combined post- and pre-capillary PH
The use of PDE5is in patients with HFpEF and isolated post-capillary PH is not
III C
recommended
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
Grupo III: HP asociada a enfermedad pulmonar
y/o hipoxia

 EPOC y/o enfisema

 Enf pulmonar intersticial
 Combinación de Fibrosis
pulmonar y enfisema
(CFPE)
 Síndromes
hipoventilatorios
 Hipoxia por altitud
elevada
 Diagnóstico

Claves diagnósticas: Indicaciones CCD:

 Presencia de factores de riesgo  Evaluación tto quirúrgico
de PAH, CTEPH o LHD  Trasplante pulmonar
 Características clínicas  Cirugía de reducción
(trayectoria de la enfermedad)  Sospecha de HAP o HPTEC
 TFP, DLCO y GSA  Si ayuda para decisiones dx o
 NT-proBNP, ECG y tto
ecocardiografía
 Imágenes transversales con Tc
con contraste, SPECT o
gammagrafía pulmonar V/Q
+/-cMRI para evaluar la
necesidad de CCD.
 Manejo
Recommendations Class Level
**If PH is suspected in patients with lung disease, it is recommended that echography be
performed and the results interpreted in conjunction with ABG, PFTs including DLCO, and CT I C
imaging
**In patients with lung disease and suspected PH, it is recommended to optimize treatment of
the underlying lung disease and, where indicated, hypoxaemia, sleep-disordered breathing, I C
and/or alveolar hypoventilation
**In patients with lung disease and suspected severe PH, or where there is uncertainty
regarding the treatment of PH, referral to a PH centre is recommended I C

*In patients with lung disease and severe PH, an individualized approach to treatment is
recommended I C

*It is recommended to refer eligible patients with lung disease and PH for LTx evaluation I C
**In patients with lung disease and suspected PH, RHC is recommended if the results are
I C
expected to aid management decisions
*Inhaled treprostinil may be considered in patients with PH associated with ILD IIb B
*The use of ambrisentan is not recommended in patients with PH associated with IPF III B
*The use of riociguat is not recommended in patients with PH associated with IIP III B
The use of PAH medication is not recommended in patients with lung disease and non-severe
PH III C
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
Grupo IV: HP tromboembólica crónica (CTEPH)

EPTEC + HP → HPTEC
Otras causas de obstrucción en AP:
Considerar HPTEC en TE:  Sarcoma de AP
 Signos Rx compatibles en  Neoplasias malignas (renal,
AngioTC útero, cs germinales en
 Defectos de llenado testículo)
 Membranas o bandas en las AP  Neoplasias no maligas
 Retracción/dilatación de la AP (leiomioma uterino)
 Perfusión en mosaico  Arteritis sin ETC
 Aumento de tamaño de arterias
bronquiales.  Estenosis de AP
 Disnea o limitación funcional  Parásitos (quiste hidatídico)
persistente post-TE  Embolismo por cuerpo extraño
 Ptes asintomáticos con FR para
HPTEC o puntuación de
predicción alta
Diagnóstico
Tratamiento
Recommendations Class Level

Manejo
**Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH
*Antiphospholipid syndrome testing is recommended in patients with CTEPH
I
I
C
C
*In patients with CTEPH and antiphospholipid syndrome, anticoagulation with VKAs is
I C
recommended
**It is recommended that all patients with CTEPH are reviewed by a CTEPH team for the
assessment of multimodality management I C
**PEA is recommended as the treatment of choice for patients with CTEPH and fibrotic
obstructions within pulmonary arteries accessible by surgery I B
**BPA is recommended in patients who are technically inoperable or have residual PH after
PEA and distal obstructions amenable to BPA I B
**Riociguat is recommended for symptomatic patients with inoperable CTEPH or
persistent/recurrent PH after PEA I B
*Long-term follow-up is recommended after PEA and BPA, as well as for patients with CTEPH
established on medical therapy I C
*A multimodality approach should be considered for patients with persistent PH after PEA and
for patients with inoperable CTEPH IIa C
*Treprostinil s.c. may be considered in patients in WHO-FC III–IV who have inoperable CTEPH
or persistent/recurrent PH after PEA IIb B
**Off-label use of drugs approved for PAH may be considered in symptomatic patients who have
inoperable CTEPH IIb B
*In patients with inoperable CTEPH, a combination of sGC stimulator/PDE5i, ERA, or
parenteral prostacyclin analogues may be considered IIb C
*BPA may be considered for technically operable patients with a high proportion of distal
disease and an unfavourable risk:benefit ratio for PEA IIb C
In patients with CTEPH who are candidates for BPA, medical therapy should be considered
prior to the intervention IIa B
 Clasificación clínica
GRUPO 1 Hipertensión arterial pulmonar (HAP)
 1.1 Idiopático GRUPO 3 PH asociado a enfermedades pulmonares y/o
 1.1.1 No respondedores a las pruebas de vasorreactividad hipoxia
 1.1.2 Respondedores agudos en las pruebas de  3.1 Enfermedad pulmonar obstructiva o enfisema
vasorreactividad  3.2 Enfermedad pulmonar restrictiva
 1.2 Hereditario  3.3 Enfermedad pulmonar con patrón mixto
 1.3 Asociado con drogas y toxinas restrictivo/obstructivo
 1.4 Asociado con:  3.4 Síndromes de hipoventilación
 1.4.1 Enfermedad del tejido conjuntivo  3.5 Hipoxia sin enfermedad pulmonar (por ejemplo, gran
 1.4.2 Infección por VIH altitud)
 1.4.3 Hipertensión portal  3.6 Trastornos pulmonares del desarrollo
 1.4.4 Cardiopatías congénitas
 1.4.5 Esquistosomiasis GRUPO 4 PH asociada a obstrucciones de la arteria
 1.5 PAH con características de afectación venosa/capilar pulmonar
(PVOD/PCH)  4.1 HP tromboembólica crónica
 1.6 PH persistente del recién nacido  4.2 Otras obstrucciones de la arteria pulmonar

GRUPO 2 PH asociada a cardiopatía izquierda GRUPO 5 PH con mecanismos poco claros y/o
 2.1 Insuficiencia cardíaca: multifactoriales
 2.1.1 con fracción de eyección preservada  5.1 Trastornos hematológicos
 2.1.2 con fracción de eyección reducida o levemente  5.2 Trastornos sistémicos
reducida 5.3 Trastornos metabólicos

 2.2 Cardiopatía valvular
 5.4 Insuficiencia renal crónica con o sin hemodiálisis
 2.3 Condiciones cardiovasculares congénitas/adquiridas
que conducen a HP postcapilar  5.5 Microangiopatía trombótica tumoral pulmonar
 5.6 Mediastinitis fibrosante
 Grupo V: HP multifactorial/mecanismo poco
claro
Trastornos asociados con la hipertensión pulmonar
1 Trastornos hematológicos •Anemia hemolítica crónica hereditaria y adquirida
•Enfermedad de célula falciforme
•β-talasemia
•Esferocitosis
•Estomatocitosis
•Trastornos autoinmunes
•Trastornos mieloproliferativos crónicos
•Leucemia mielógena crónica
•Policitemia vera
•Mielofibrosis idiopática
•Trombocitopenia esencial
•Otros
2 Trastornos sistémicos Sarcoidosis
Pulmonar Histiocitosis de células de Langerhans
Neurofibromatosis tipo 1
3 Trastornos metabólicos Enfermedad de almacenamiento de glucógeno
Enfermedad de Gaucher
4 Insuficiencia renal crónica con/sin hemodiálisis
5 Microangiopatía trombótica tumoral pulmonar
6 Fibrosis mediastinitis
Resumen
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