Show notes
CardioNerds (Amit Goyal & Karan Desai) join University of Minnesota fellows, Dr. Julie Power, Dr. Sasha Prisco, and Dr. Abdisamad Ibrahim for a riveting discussion in which they were pressured to diagnose a young woman with syncope. The fellows expertly take us through the next steps in the differential diagnosis, and management of pulmonary hypertension in this young patient! University of Minnesota faculty and expert in right ventricular (RV) failure in pulmonary arterial hypertension (PAH) Dr. Kurt Prins provides the E-CPR for this episode.
With this episode, the CardioNerds family warmly welcomes The University of Minnesota to the CardioNerds Healy Honor Roll. The CardioNerds Healy Honor Roll programs support and foster the the CardioNerds spirit and mission of democratizing cardiovascular education. Healy Honor Roll programs nominate fellows from their program who are highly motivated and are passionate about medical education. The University of Minnesota fellowship program director, Dr. Jane Chen has nominated Dr. Julie Power for this position. In addition to being a CardioNerds Ambassador, Julie has already done amazing CardioNerds work as part of the CardioNerds Academy fellowship.
Claim free CME just for enjoying this episode! Disclosures: None
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Patient Summary- Syncope and Pulmonary Hypertension
A Somali woman in her mid-30s with no significant past medical history presented with shortness of breath and exertional syncope. EKG revealed evidence of RV strain. CTA-PE protocol did not show PE. However, there was RV dilation and subsequent echocardiogram demonstrated normal LV, but moderately reduced RV function with evidence of RV pressure and volume overload. RVSP was estimated to be 188 mmHg!
Case Media
ABCDEFGHClick to Enlarge
A. CXR, B. ECG, C. PA measurements: Main PA measures 2.4 cm, right PA measures 2.3 cm, left PA measures 1.9 cm, D. Tricuspid valve Doppler, E. RA tracing, F. RV tracing, G. PA tracing, H. Wedge tracing
CTA PE: No PE, markedly dilated pulmonary trunk at 4.7 cm. Right main pulmonary artery measures 3.1 cm.
TTE: Parasternal long axis: Moderate right ventricular dilation compressing left ventricle. Global right ventricular function is moderately reduced.
TTE: Parasternal long axis- RV view: Right ventricular dilation with mild pulmonary regurgitation
TTE: Mild pulmonary regurgitation with dilation of main PA
TTE: Paradoxical septal motion consistent with right ventricular pressure and volume overload.
TTE: Apical 4 chamberParadoxical septal motion consistent with right ventricular pressure and volume overload. Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement.
Paradoxical septal motion consistent with right ventricular pressure and volume overload.Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement.Moderate to severe tricuspid regurgitation.
TTE: Positive bubble study
Episode Teaching
Pearls
Pulmonary hypertension (PH) can generally be categorized as pre-, post-, or combined pre- and post-capillary PH. Isolated pre-capillary pulmonary hypertension is characterized by: mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) ≥ 3 Woods units (WU). Pulmonary arterial hypertension (PAH) (WHO Group 1) falls under pre-capillary pulmonary hypertension.Schistosomiasis is the most common cause of PAH (WHO Group I) worldwide. Approximately 7% of patient with hepatosplenic schistosomiasis have PAH. Some studies suggest that treatment of with praziquantel reverses vascular remodeling; however, there is point of no return, beyond which, anthelmintic therapies are ineffective to prevent progression.Exertional syncope and pericardial effusion are both risk factors for higher mortality in PAH.Women with severe PAH have extremely high risk of maternal morbidity and mortality. Endothelin receptor antagonists are contraindicated in pregnancy due to teratogenicity. Therefore, a pregnancy test must be obtained monthly while on this therapy.Patients with a lower socioeconomic status, based on median household income, have more advanced PAH at the time of diagnosis.
Notes
1. How do you approach syncope?
Syncope is a sudden transient loss of consciousness associated with absence of postural tone followed by complete and usually rapid recovery. There should be not be clinical evidence of “non-syncope” conditions including seizures, hypoglycemia, drug or alcohol intoxication, concussion due to head trauma and so forth.
One approach to determining the etiology of the syncope is to consider 4 major categories: orthostatic, reflex-mediated, cardiac-obstructive, or cardiac-electrical.Reflex-mediated (neurocardiogenic) syncope typically has a prodrome and encompasses vasovagal syncope, situational syncope, and carotid hypersensitivity.Orthostatic syncope is syncope occurring when rising from recumbency. It is generally associated with an orthostatic SBP drop by more than 20 mmHg or DBP drop by more than 10 mmHg with a compensatory rise in heart rate. We most commonly think of dehydration or hypovolemia causing orthostasis. Being post prandial can cause orthostasis as well. Neurogenic orthostatic hypotension (OH) involves excessive pooling of blood volume in the splanchnic and/or leg circulation. Upon standing, there is decreased venous return to the heart with a subsequent decrease in cardiac output and cerebral perfusion. The autonomic nervous system can typically increase vascular tone, inotropy and chronotropy; however, in neurogenic OH these mechanisms are inadequate. Conditions where neurogenic OH is relatively common include multiple system atrophy, Parkinson’s disease, Huntington’s disease, peripheral neuropathies (e.g., diabetes, amyloidosis), and spinal cord injury, amongst other etiologies. Finally, common medications associated with orthostatic syncope include diuretics, alpha blockers, and tricyclic antidepressants.Cardiac-obstructive syncope may occur from structural obstruction (i.e., aortic stenosis, HCM, mitral stenosis, pulmonary embolism) or other lesions which limit the stroke volume (i.e., pericardial tamponade, pulmonary hypertension.Cardiac-electrical syncope include both tachyarrhythmias and bradyarrhythmias, often without a prodrome.
2. What are the different types of pulmonary hypertension (PH)? What are the hemodynamic definitions of pulmonary hypertension?
The WHO separates PH into 5 groups:Group 1: Pulmonary arterial hypertension (e.g., idiopathic, heritable [BMPR2], anorexigen associated, drug or toxin-associated, HIV, connective tissue disease associated, schistosomiasis, portal hypertension, congenital heart disease, amongst other causes)Group 2: Pulmonary hypertension due to left sided heart disease (e.g., HFrEF, HFpEF, left-side valvular heart disease)Group 3: Pulmonary hypertension due to lung disease or hypoxia: (e.g.,COPD, ILD, OSA, hypoxia without lung disease such as high altitude, developmental lung disorders)Group 4: PH due to pulmonary artery obstructions most commonly Chronic Thromboembolic Pulmonary Hypertension (CTEPH)Group 5: Multifactorial 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)
When we consider the hemodynamics of pulmonary hypertension, we break down PH into isolated pre-capillary, isolated post-capillary, or combined pre-and post-capillary pulmonary hypertension.
Mean Pulmonary Artery Pressure (mmHg)Wedge Pressure (mmHg)Pulmonary Vascular Resistance (Woods Units)WHO GroupsPre-capillary 20≤ 15≥ 31, 3, 4, 5Post-capillary 20 15 20 15≥ 32, 5, multifactorial
PAH falls under pre-capillary pulmonary hypertension, which is defined as mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) ≥ 3 Woods units (WU).
3. How do you work up suspected PAH?
To start investigating for PAH, as always, we start with a thorough history and physical. The most common presenting symptom of pulmonary hypertension in general is exertional dyspnea/reduced exercise tolerance. Symptoms of PH can be nonspecific, especially early in its course, and thus there can be a delay in diagnosis. Remember, some patients have increased risk of developing PH and should be screened. These are patients with known risk factors for developing PAH, including relatives of patients with BMPR2 mutations, HIV, connective tissue disease (especially systemic sclerosis), portal hypertension, etc.Other accompanying symptoms may include chest pain, fatigue, and lightheadedness. Manifestations of more advanced disease include syncope, abdominal distension, and significant lower extremity edema attributable to right ventricular (RV) failure.Physical exam may reveal a loud P2, murmur of tricuspid regurgitation, an RV S3, jugular venous distension with or without Kussmaul’s sign, liver pulsatility, ascites, and/or peripheral edema.Review the CXR and EKG for findings consistent with pulmonary hypertension. Common EKG findings include right atrial enlargement, right axis deviation, RBBB, and an RV strain pattern in the right precordial leads.