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CardioNerds (Amit Goyal and Daniel Ambinder) join Dr. Kushani Gajjar and Dr. Mitha Naik from the Allegheny Health Network for a walk along the Three Rivers Trail in Pittsburgh. They discuss a case of young woman in her third trimester of pregnancy with a known history of pulmonary arterial hypertension. The management of pulmonary hypertension in pregnancy and RV failure in the context of pregnancy is described. The E-CPR segment is provided by Dr. Nandita Scott, Co-Director Corrigan Women's Heart Health Program and Cardiovascular Disease and Pregnancy Service at Massachusetts General Hospital. Special cameo appearance by Dr. Dani Crousillat.
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Patient Summary - Pulmonary Hypertension in Pregnancy
A 33-year-old woman in the third trimester of pregnancy, with a known history of untreated PAH in the setting of TKI therapy, presents with shortness of breath. She is found to have PA pressure greater than systemic pressure with PASP 130. We describe the management of PH and RV failure in the context of a pregnancy. The patient was admitted to the ICU where a multidisciplinary team was mobilized, involving high risk ob-gyn, maternal fetal medicine, critical care, anesthesiology, and advanced heart failure. They began pulmonary vasodilators including treprostinil, tadalafil and inhaled nitric oxide. They also added inotropic and vasopressor support for right ventricular dysfunction with her severe PAH. Fetal heart monitoring was performed. PAH also led to worsening of known chronic thrombocytopenia in the setting of CML. HELLP syndrome was ruled out. The patient had preterm rupture of membranes at 32 weeks of gestation and the team pursued assisted vaginal delivery to prevent vagal response. Following successful delivery, the patient elected to undergo intra-uterine device placement. Post-delivery, vasopressors and inotropes were weaned, and she was discharged on treprostinil, ambrisentan and tadalafil. Thankfully both the mother and baby returned healthy and well at 1 month follow up.
Case Media - Pulmonary Hypertension in Pregnancy
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Episode Teaching - Pulmonary Hypertension in Pregnancy
Pearls - Pulmonary Hypertension in Pregnancy
Pulmonary Hypertension is defined as a mean pulmonary arterial pressure (mPAP) of 20mmHg. There are 5 major types of pulmonary hypertension. Risk modifiers include symptom burden, exercise capacity, presence of pericardial effusion, RV function and hemodynamics.Multidisciplinary care teams are the key to achieving optimal pregnancy outcomes in patients with PH. It is critical to create a team of experts with experience in pulmonary hypertension and plan for constant communication before, during, and after pregnancy.Pregnant women who are already on PAH therapy outpatient should continue them during pregnancy (under the direction of PH experts) except for endothelin receptor blockers which fall in pregnancy category X.PH during pregnancy is associated with up to 38% maternal mortality rate as the right ventricle (RV) is often unable to handle the volume shifts and hemodynamic changes that occur during pregnancy, labor, and delivery.Patients with RV failure leading to low cardiac output and hypotension, like in this case, may benefit from vasopressors with the goal to maintain systemic blood pressure above pulmonary arterial pressures, preserving right coronary blood flow and preventing intracardiac shunting. Swan-guided management may be useful - to titrate inotropes and vasopressors, optimize pulmonary vasodilators and ensure that the patient is being adequately fluid-optimized to maintain a CVP of around 8-10.
Notes - Pulmonary Hypertension in Pregnancy
1. What are the different types of pulmonary hypertension (PH)?The WHO separates PH into 5 groups:
Group 1Pulmonary arterial hypertension (e.g., idiopathic, heritable [BMPR2], anorexigen associated, drug or toxin-associated, HIV, connective tissue disease associated, schistosomiasis, portal hypertension, congenital heart disease, etc) Group 2Pulmonary hypertension due to left sided heart disease (e.g., HFrEF, HFpEF, left-side valvular heart disease) Group 3Pulmonary hypertension due to lung disease or hypoxia: (e.g., COPD, ILD, OSA, hypoxia without lung disease such as high altitude, developmental lung disorders) Group 4PH due to pulmonary artery obstructions most commonly Chronic Thromboembolic Pulmonary Hypertension (CTEPH)Group 5Multifactorial causes such as hematologic disorders (chronic hemolytic anemia, as with myeloproliferative disorders), metabolic disorders (e.g., Gaucher disease, glycogen storage diseases, CKD), and systemic disorders (e.g., pulmonary Langerhans cell histiocytosis, neurofibromatosis, sarcoidosis)
2. What is the role of TTE and RHC in the workup of PH?
Tyrosine kinase inhibitors are known to cause PAH and there should be a low threshold to do screening TTEs in these patients when there is concern for the development of PAH.Echocardiogram is essential in the evaluation of PH –for positing the presence of PH, evaluating the left heart and congenital defects for clues to PH etiology, and assessing the structural consequences of PH on the right heart. In addition to estimating the pulmonary artery systolic pressure (PASP) by measuring the tricuspid regurgitant jet, we can characterize RV and RA size, RV function, and RV wall thickness, which may help both support the diagnosis and gauge prognosis for PH. Further, we can evaluate for left-sided heart disease contributing to PH. The presence of a pericardial effusion is a poor prognostic sign in those with pulmonary hypertension. Notably, the PASP is not of prognostic value due both to inaccuracies in measurement and falling values in the context of severe PH with failing RV function; a such PASP should not be used to surveil patients with PH or monitor response to PH therapies.
McLaughlin et al suggest using a checklist in the echocardiographic assessment of PH which includes the following1:
ChecklistSalient pointsEstimated PASPTo calculate mean PAP, usually PA diastolic pressure needs to be assessed which often requires a PR jet which is both difficult to obtain and not often present.Evaluate RV size and functionSize: abnormal RV basal diameter 4.2 cm, wall thickness in subcostal view 5 mm Function: TAPSE (abnormal 20≤ 15≥ 31, 3, 4, 5Post-capillary 20 15 20 15≥ 32, 5, multifactorial
Furthermore, we can determine if a patient is “vasodilator responsive.” In the catheterization lab, a positive vasodilator response is defined as a decrease in mPAP ≥ 10 mmHg to an absolute value of ≤ 40 mmHg (without a decrease in cardiac output) with the use of inhaled nitric oxide or IV epoprostenol. If a patient has positive vasodilator test, calcium channel blockers can be initiated, however not all patients will be long term responders. We tend to do vasoreactivity testing in patients with PAH and not for other forms of PH (e.g., Pulmonary Veno-Occlusive Disease or Groups 2, 3, 4, or 5).
3. What are PAH-specific pharmacologic treatments?
Remember that PAH is fundamentally a disease of increased pulmonary vascular resistance (PVR) causing elevated pulmonary pressures. The consequence of increased PVR includes increased RV afterload and hypoxemia and the subsequent clinical manifestations of PAH. Normally, the pulmonary vascular bed has a balance between vasodilators and vasoconstrictors that maintain a low-resistance, high-compliance state. This balance is disturbed in PAH and the goal of therapy is to “restore” balance between vasodilation and vasoconstriction.The management of PAH relies on 3 primary medication groups1: