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__NOTOC__
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{{Pulmonary hypertension}}
{{Pulmonary hypertension}}
{{CMG}}  '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]]
{{CMG}}  '''Assistant Editor(s)-in-Chief:''' {{Jose}}; [[User:Ralph Matar|Ralph Matar]]
==Overview==
==Overview==


Patients with a known [[BMPR2]] [[mutation]], [[scleroderma]], and [[portal hypertension]] undergoing evaluation for [[liver transplantation]] should receive periodic screening by means of [[echocardiography]]. Evaluation for thromboembolic disease is also appropriate in all patients suspected of having pulmonary hypertension.<ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>
Patients with a known [[BMPR2]] [[mutation]], [[scleroderma]], and [[portal hypertension]] undergoing evaluation for [[liver transplantation]] should receive periodic screening for pulmonary hypertension (PH) through [[echocardiography]].  


==Screening==
==Screening==
Patients who are considered at high risk for the development of pulmonary hypertension and require periodic screening include:  
* Studies have not shown an impact on outcomes with [[pulmonary hypertension]] [[screening]];
*Those with a known BMPR2 [[mutation]](Associated with Familial Pulmonary Hypertension).
* Despite that, there is an expert consensus that some groups of patients must be screened for [[pulmonary hypertension]] such as:
*Those with [[Scleroderma]] spectrum of diseases.
**Patients with [[scleroderma]] spectrum disorders (especially the ones with corrected [[DLCO]] less than 80%);
*Those with [[portal hypertension]] who are undergoing evaluation for [[liver transplantation]].
**Patients with mutations for a heritable form of [[PAH]];
**Patients with portal hypertension being considered for organ transplantation;
* These patients must be screened annually with [[echocardiography]].<ref name="pmid33844574">{{cite journal| author=Poch D, Mandel J| title=Pulmonary Hypertension. | journal=Ann Intern Med | year= 2021 | volume= 174 | issue= 4 | pages= ITC49-ITC64 | pmid=33844574 | doi=10.7326/AITC202104200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33844574  }} </ref>


The best study for screening patients suspected of having pulmonary hypertension based on history, [[physical examination]], [[chest x-ray]] (CXR), and [[electrocardiogram]] (ECG) is an '''[[Echocardiogram]]'''.
===Summary===
Shown below is a table summarizing the recommended screening in several medical conditions associated with elevated risk for PH.<ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref><ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>


These are the parameters that should be evaluated on [[Echocardiogram]]:
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
#Right atrial and right ventricular enlargement.
|-
#Reduced right ventricular function.
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 40%" align="center" |'''Condition''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 60%" align="center" |'''Recommended screening'''
#Displacement of the [[interventricular septum]].
|-
#[[Tricuspid regurgitation]](TR).
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |''' Known [[BMPR2]] [[mutation]]''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] (yearly)
#Tei index or [[myocardial]] performance index (measured by [[Doppler ultrasound]]).
|-
#[[Pericardial effusion]] (presence also indicates a higher mortality rate).
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''BMPR2 [[mutation]] in a first degree relative''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Genetic counseling]] <br> [[BMPR2]] genotyping
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''Family history for PAH in 2 or more relatives'''|| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" | [[Genetic counseling]] <br> [[BMPR2]] genotyping
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''[[Systemic sclerosis]]'''|| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] (yearly)
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''[[Portal hypertension]]''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] if orthotopic liver transplantation is in consideration
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''[[Sickle cell disease]]''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] (yearly)
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''Previous use of [[fenfluramine]]''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] in case of symptoms
|-
| style="font-size: 100; padding: 0 5px; background: #DCDCDC" align="left" |'''[[Congenital heart disease]]''' || style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |[[Echocardiogram]] at the time of diagnosis
|}


The Tei index is an index of combined right ventricular [[systolic]] and [[diastolic]] function obtained by dividing the sum of both isovolumetric contraction and relaxation intervals by the ejection time.
===Echocardiography findings===
[[Echocardiography]] findings that are suggestive of PH include:<ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>
# Enlargement of the size of [[right atrium]] and [[right ventricle]]
# Decrease in the function of the [[right ventricle]]
# Displacement of the [[interventricular septum]]
# [[Tricuspid regurgitation]]
# Presence of [[pericardial effusion]]


==References==
==References==
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Latest revision as of 17:34, 9 June 2021

Pulmonary Hypertension Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: José Eduardo Riceto Loyola Junior, M.D.[2]; Ralph Matar

Overview

Patients with a known BMPR2 mutation, scleroderma, and portal hypertension undergoing evaluation for liver transplantation should receive periodic screening for pulmonary hypertension (PH) through echocardiography.

Screening

  • Studies have not shown an impact on outcomes with pulmonary hypertension screening;
  • Despite that, there is an expert consensus that some groups of patients must be screened for pulmonary hypertension such as:
    • Patients with scleroderma spectrum disorders (especially the ones with corrected DLCO less than 80%);
    • Patients with mutations for a heritable form of PAH;
    • Patients with portal hypertension being considered for organ transplantation;
  • These patients must be screened annually with echocardiography.[1]

Summary

Shown below is a table summarizing the recommended screening in several medical conditions associated with elevated risk for PH.[2][3]

Condition Recommended screening
Known BMPR2 mutation Echocardiogram (yearly)
BMPR2 mutation in a first degree relative Genetic counseling
BMPR2 genotyping
Family history for PAH in 2 or more relatives Genetic counseling
BMPR2 genotyping
Systemic sclerosis Echocardiogram (yearly)
Portal hypertension Echocardiogram if orthotopic liver transplantation is in consideration
Sickle cell disease Echocardiogram (yearly)
Previous use of fenfluramine Echocardiogram in case of symptoms
Congenital heart disease Echocardiogram at the time of diagnosis

Echocardiography findings

Echocardiography findings that are suggestive of PH include:[4]

  1. Enlargement of the size of right atrium and right ventricle
  2. Decrease in the function of the right ventricle
  3. Displacement of the interventricular septum
  4. Tricuspid regurgitation
  5. Presence of pericardial effusion

References

  1. ↑ Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check |pmid= value (help).
  2. ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
  3. ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
  4. ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension

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