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Pulmonary Hypertension in Systemic Disease

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Luke Howard

on 22 May 2015

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Transcript of Pulmonary Hypertension in Systemic Disease

Pulmonary Hypertension in Systemic Disease
Classification defines treatment
PH is classified into one of five categories
1. Pulmonary arterial hypertension (PAH)
1’. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
2. PH due to left heart disease
3. PH due to lung disease and/or hypoxaemia
4. Chronic thromboembolic PH (CTEPH)
5. PH with unclear and/or multifactorial mechanisms

Idiopathic PAH "Primary"
Heritable PAH
BMPR2
ALK-1, ENG, SMAD9, CAV1, KCNK3
Unknown
Drugs or toxins induced
PAH associated with:
Connective tissue disease
HIV
Portal hypertension
Congenital heart disease
Schistosomiasis
PAH Subclassification
Simonneau G, et al. J Am Coll Cardiol 2013;62:D34–D41

*
*
*

*
* Extrapulmonary disorders causing pulmonary vascular remodelling similar to IPAH
Group 5 subclassification
5.1 Haematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy

5.2 Systemic disorders: sarcoidosis, pulmonary histiocytosis,
lymphangioleiomyomatosis

5.3Metabolic disorders: glycoge nstorage disease, Gaucher disease, thyroid disorders

5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH
"Pre-capillary PH"
Pre-Capillary Pulmonary Hypertension
1. PAH
3. PH due to lung disease and/or hypoxaemia
4. Chronic thromboembolic PH
5. PH with multifactorial and/or unclear mechanisms
Pathophysiology
Mean pulmonary artery pressure (mPAP) > or = 25 mmHg

Pulmonary wedge pressure (PWP), left atrial pressure (LAP) or left ventricular end-diastolic pressure (LVEDP) < or = 15 mmHg

PVR > 3 Wood units
Haemodynamics
Increase in pulmonary vascular resistance due to pulmonary arterial:
remodelling,
narrowing,
destruction,
and/or
occlusion.
Hoeper M, et al J Am Coll Cardiol 2013;62:D42–50
Functional imaging assessment
Echocardiography
Cardiac magnetic resonance
Pulmonary Hypertension Diagnosis
Accurate diagnosis and classification requires a multimodality approach
Static imaging
Pulmonary angiography
Ventilation-perfusion scan
CT and X-Ray
Ultrasound
Physiological assessment
Exercise testing
Pulmonary function
Right heart catheterisation
Clinical assessment
History & examination
Functional Class

Bloods
Diagnosis
Echocardiography
Diagnosis
Suspecting PH
Identifying causes of PH

Assessment
Severity of PH
Severity of right ventricular impairment
Response to therapy
Dr Luke Howard, 2014
Idiopathic PAH
Heritable PAH
BMPR2
ALK-1, ENG, SMAD9, CAV1, KCNK3
Unknown
Drugs or toxins induced
PAH associated with:
Connective tissue disease
HIV
Portal hypertension
Congenital heart disease
Schistosomiasis
PAH Subclassification
*
*
*

*
* Extrapulmonary disorders causing pulmonary vascular remodelling similar to IPAH
Dr Luke Howard
National Pulmonary Hypertension Service (London)
Hammersmith Hospital &
Imperial College London

CTD-aPAH
Scleroderma APAH
Prevalence 7-12%
Rationale for annual screening in asymptomatic patients with lung function and echocardiography
? only in those with DLCO < 55-60%
Historically poorer survival
Older
More left ventricular disease
Worse right ventricular function for same PH
Pulmonary venous involvement
Interstitial lung disease
Multisystem disease
Connective Tissue Disease
Connective Tissue Disease - APAH
Scleroderma
Limited, anti-centromere antibodies, many years after diagnosis, primary pulmonary vasculopathy
Diffuse, Scl70, aggressive disease, secondary to fibrotic lung disease, left ventricular disease
Mixed Connective Tissue Disease
Systemic Lupus Erythematosus
Sjogrens
Dermatomyositis
Rheumatoid Arthritis
Immunosuppression
Earlier Diagnosis ? Better outcomes
Idiopathic/heritable: 504 patients, median age 66 y
Connective tissue disease: 382 patients, median age 65 y
Congenital heart disease 278 patients, median age 39 y
UK Survival Data
Olsson et al., Circ 2013
Unlike in IPAH, my personal practice is not to anticoagulate CTD-APAH....
Prevalence 0.5%

Prior to highly active antiretroviral therapy (HAART) and PAH drugs
mortality 50% at 1 year

Now
70% 5 year survival with HAART and PAH drugs
~20% normalise haemodynamics
HIV-APAH
Portopulmonary Hypertension
Definitive treatment is liver transplantation
Aim for mPAP < 35 mmHg (normal < 20)
and
low resistance < 3 Wood Units (normal ~1)

Beta blockers worsen exercise capacity
Group 5 subclassification
5.1 Haematologic disorders:
chronic hemolytic anemia*
myeloproliferative disorders*
splenectomy
5.2 Systemic disorders:
sarcoidosis*
pulmonary histiocytosis
lymphangioleiomyomatosis
General Principle: Treat the Underlying Disease

5.3Metabolic disorders:
glycogen storage disease
Gaucher disease
thyroid disorders
5.4 Others:
tumoral obstruction
fibrosing mediastinitis
chronic renal failure*
segmental PH
Sickle Cell Disease
Sickle lung disease
Pulmonary infarction

Fibrotic lung disease

Restrictive lung function
Pulmonary vascular disease
Thromboembolic pulmonary hypertension

"Pulmonary arterial hypertension"-like disease
High output heart failure
Left ventricular disease
Post-capillary PH
Myeloproliferative Disorders
Aggressive treatment of underlying disease
Chemotherapy
Caution - may cause PH (some TKis)
May reverse PH
Total lung irradiation

Consider treatment with PAH therapies
Sarcoidosis
Immunosuppression
Prednisolone
Methotrexate
Azathioprine
Mycophenolate mofetil

PAH therapies


Stage 0 >> 4
Pulmonary Hypertension related to Renal Failure
Left ventricular dysfunction
Fluid overload
Left ventricular dysfunction
Fluid overload
Left ventricular dysfunction
Fluid overload
Left ventricular dysfunction
Fluid overload
Left ventricular dysfunction
Fluid overload
Pulmonary hypertension due to end-stage renal failure on haemodialysis
Undertake cardiac catheterisation assessment just after haemodialysis and use a fluid challenge to assess LV function
Schistosomiasis PAH
Many shared vascular features with IPAH

Treatment:
Eradication of parasite
1-2 courses of Praziquantel
If persistent PAH, treatment with PAH therapies
Baughman et al., Chest 2014
Baughman et al., Chest 2014
Baughman et al., Chest 2014
Pressure = flow x resistance + downstream pressure
Pulmonary Vasoactive Treatment
GSK Data on File*
Galie et al., JACC 2013
McLaughlin et al.,
JACC 2013
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