Journal of the Practice of Cardiovascular Sciences

CURRICULUM IN CARDIOLOGY - CASE DISCUSSION
Year
: 2018  |  Volume : 4  |  Issue : 2  |  Page : 126--131

Pulmonary arterial hypertension with frequent syncope: Clinical case presentation


Aseem Basha, Shanmugam Krishnan, Jaskaran Gujral, KC Goswami 
 Department of Cardiology, AIIMS, New Delhi, India

Correspondence Address:
Dr. Aseem Basha
Department of Cardiology, AIIMS, New Delhi
India

Abstract

Pulmonary arterial hypertension presenting with frequent syncope has been highlighted in this bedside case discussion. The clinical approach to such a patient, including review of the ecg, chest radiograph and echocardiogram is presented. Various aspects of the bedside approach to adult cyanotic heart disease are discussed. Special emphasis on management strategies in pulmonary arterial hypertension have also been discussed.



How to cite this article:
Basha A, Krishnan S, Gujral J, Goswami K C. Pulmonary arterial hypertension with frequent syncope: Clinical case presentation.J Pract Cardiovasc Sci 2018;4:126-131


How to cite this URL:
Basha A, Krishnan S, Gujral J, Goswami K C. Pulmonary arterial hypertension with frequent syncope: Clinical case presentation. J Pract Cardiovasc Sci [serial online] 2018 [cited 2020 Jan 20 ];4:126-131
Available from: http://www.j-pcs.org/text.asp?2018/4/2/126/240966


Full Text

 Clinical Presentation



A 31-year-old female, a resident of Uttar Pradesh, India, presented with complaints of:

Shortness of breath on exertion for 4 monthsThree episodes of syncope in the last 3 months.

She was apparently well 4 months back when she developed dyspnea on exertion, which had progressed from NYHA class I at the onset to NYHA class II over 4 months. During the last 4 months, she had three episodes of syncope. She had these three episodes of loss of consciousness while shopping and climbing stairs. Each episode occurred following exertion and was associated with loss of postural tone, transient blackout, and jerky movements of limbs followed by complete recovery in 5–10 min. There was no postictal drowsiness or focal deficits after the event. There was no history of chest pain, palpitations, cyanosis, clubbing, alcohol abuse, and drug intake. Her medical history was significant for hypothyroidism for which she was on thyroid supplements, and she was nondiabetic and nonhypertensive. She underwent a medical termination of pregnancy (MTP) a month prior to her onset of symptoms. There was no other significant medical/surgical or psychiatric illness. Family history was noncontributory. Her three previous pregnancies were uneventful.

 Summary at the End of History



The case was a 31-year-old female symptomatic for 4 months in the form of progressive dyspnea on exertion with three episodes of exertional syncope in the last 3 months.

 Discussion



Is it seizure or syncope?

The events described seem to favor syncope over seizures. All the episodes were exertional. The jerky movements during syncope differ from those accompanying a grand mal epileptic seizure, which are described as follows:

Would be briefer in patients with syncopeUsually occur after the loss of consciousness has set inUsually do not have the symmetric tonic-clonic features of a grand mal epileptic seizure.

Furthermore, seizure may be preceded by an aura, associated with automatism, of relatively prolonged duration, and usually succeeded by postictal drowsiness and confusion.

What is the first differentia diagnosis on the basis ofyour history?

The first differential diagnosis would be an obstructive valve lesion such as aortic stenosis or hypertrophic cardiomyopathy in view of progressive dyspnea and exertional syncope.

What are your differential diagnoses?

In addition to obstructive lesions such as aortic stenosis, hypertrophic obstructive cardiomyopathy, tachyarrhythmias, bradyarrhythmias, tetralogy of Fallot, and severe pulmonary arterial hypertension (PAH) should also be considered in the differentials.

Hypertrophic obstructive cardiomyopathy

Hypertrophic obstructive cardiomyopathy is the most important cause of self-terminating ventricular arrhythmias, which can also present with shortness of breath and syncope and would be the second differential based on the history.

Pulmonary hypertension

A young lady with complaints of shortness of breath on exertion and syncope should also warrant an evaluation for pulmonary arterial hypertension. In addition, a history of MTP prior to the onset of symptoms may also point toward the possibility of thromboembolic pulmonary hypertension.

Tachyarrhythmias and bradyarrhythmia

Tachyarrhythmias and bradyarrhythmia are the second most common cause of cardiac syncope; however, progressive shortness of breath in the absence of palpitation would make it less likely.

Tetralogy of Fallot

Tetralogy of Fallot can also give rise to progressive exertional dyspnea and exertional syncope. Absence of the history of cyanosis and clubbing, however, makes it unlikely.

Eisenmenger syndrome

Eisenmenger syndrome can also have a similar presentation. However, in the absence of a history of recurrent hospital admissions in early infancy, an uneventful childhood and adolescence make the possibility of Eisenmenger syndrome less likely.

How common is syncope in these conditions?

Listed below are the common cardiac causes of syncope and their relative incidence are summarized in [Table 1].{Table 1}

What are the mechanisms for syncope in the above-mentioned differential diagnoses?

Aortic stenosisSyncope on exertionReduced cerebral perfusion that occurs during exertion when arterial pressure declines consequent to systemic vasodilation in the presence of a fixed cardiac output (CO)Malfunction of the baroreceptorVasodepressor response to a greatly elevated left ventricular (LV) systolic pressure during exercise.Syncope at restSyncope at rest may be caused by transient atrial fibrillation (AF) with loss of the atrial contribution to LV filling, which causes a precipitous decline in COTransient atrioventricular block caused by extension of the calcification of the valve into the conduction system.Hypertrophic cardiomyopathy[2]ArrhythmiaParoxysmal AF or supraventricular tachycardiaComplete heart blockSinus node dysfunctionSustained ventricular tachycardiaPrimary hemodynamic mechanismLV outflow tract obstructionInappropriate vasodilation consequent to abnormal vascular control due to inappropriate vasodilationHypotension due to impaired filling when preload is decreased in the setting of diastolic dysfunctionPulmonary hypertensionLoss of ventricular adaptation to afterloadDecreased right ventricular (RV) output and RV–LV interdependenceUncoupling between RV and pulmonary circulationUnmet demand to increase CO during exerciseDrop in systemic pressure due to an abrupt decrease in stroke volumeOther contributorsArrhythmiasRV and/or LV ischemia due toSubendocardial microcirculationDynamic occlusion of left coronary artery due to PA dilatationSevere PA dilatation

 Examination



The patient was an average-built female. Pulse was 92 beats/min; regular, normal volume, no radio-radial or radio-femoral delay, and all peripheral pulses were palpable. Blood pressure was 100/70 mmHg in the left upper limb in supine position with no significant difference in upper- and lower-limb blood pressures and there was no postural drop. Her respiratory rate was 18/min, and she was afebrile at the time of presentation. Jugular venous pressure (JVP) was elevated, 6 cm above the sternal angle with prominent “a” waves. There was no pallor, icterus, cyanosis, clubbing, or lymphadenopathy. Oxygen saturation was 98% on room air at rest and exertion.

Cardiovascular examination

Inspection

Precordium was normal. Cardiac apex was visible in the left 5th intercostal space in the midclavicular line. There were no scars, sinuses, or dilated superficial veins.

Palpation

Apex beat was present in the left 5th intercostal space in the mid-clavicular line, was diffuse, and was an RV type of apex. Palpable second sound was there in the left 2nd intercostal space and palpable S4 at the left lower sternal border was present. Grade II left parasternal heave was also appreciated.

Percussion

Left cardiac border was coincident with the apex, right cardiac border was behind the sternum, and left second intercostal space was dull on percussion.

Auscultation

The first heart sound (S1) was normal. S2 was narrowly split with loud P2 component. RV S4 was heard in the left lower sternal border. There were a short ejection systolic murmur Grade II/VI heard in pulmonary area and a Grade 3/6 pansystolic murmur in the tricuspid area.

Systemic examination

Respiratory system, central nervous system, and per abdomen examination findings were within normal limits.

Summary of clinical findings

Clinical examination revealed an average-built female with elevated JVP with prominent A waves, palpable S2 with Grade II parasternal heave with RV apex, auscultatory findings of narrow split second heart sound with loud P2 component, RV S4 with a short systolic murmur being heard in pulmonary area, and a Grade 3/6 pansystolic murmur in tricuspid area.

What is your diagnosis based on history and examinationfindings?

A young female, with complaints of dyspnea and syncope, on examination having features of pulmonary hypertension in the form of elevated JVP, loud and palpable P2, Grade II parasternal heave, RV S4, and a short mid-systolic murmur without differential cyanosis all point toward the diagnosis of idiopathic PAH (IPAH).

How do you differentiate idiopathic pulmonary arterial hypertension from Eisenmenger syndrome?

The differentiation has been summarized in [Table 2].[4],[5],[6]{Table 2}

What are the investigations done in this case?

Electrocardiography (ECG) revealed sinus bradycardia with a heart rate of 55/min with T-wave inversion in V1–V3 and lead III. There was right-axis deviation. QT, QTc, and PR intervals were normal, and the ECG showed no evidence of right atrial enlargement [Figure 1].{Figure 1}

Chest X-ray posteroanterior view revealed enlarged main pulmonary, left pulmonary, and right pulmonary arteries with peripheral pruning. Lateral projection revealed RV enlargement [Figure 2] and [Figure 3].{Figure 2}{Figure 3}

Echocardiography shows dilated right artery (RA), right ventricle (RV), moderate tricuspid regurgitation (TR), severe PAH with right ventricular systolic pressure = right arterial pressure + 80 mmHg, mild PR, no atrial septal defect, ventricular septal defect, or patent ductus arteriosus. Valves were normal, and there was no clot/vegetation. Contrast echo with agitated saline was negative for any shunts [Figure 4], [Figure 5], [Figure 6].{Figure 4}{Figure 5}{Figure 6}

Computed tomography pulmonary angiogram was suggestive of PAH with dilated RA and RV with right ventricular hypertrophy. There was no evidence of chronic thromboembolic pulmonary hypertension [Figure 7]. V/Q scan was negative.{Figure 7}

Other investigations

Six-minute walk test distance was 400 m. Ultrasound abdomen and pulmonary function tests were within normal limits. Systemic lupus erythematosus workup was also negative.

Final diagnosis

Severe PAH-probably idiopathic, moderate TR 26SyncopeWHO-FC IINormal sinus rhythmNormal biventricular functionCongestive heart failureHypothyroidism.

What is your management strategy in pulmonary arterial hypertension?

There are three main steps in the management:[7]

General measures, supportive therapy, acute vasoreactivity testingInitial therapy with high-dose calcium channel blockers (CCBs) in vasoreactive patients or other drugsResponse to initial treatment strategy, combination therapy, and surgical options.

General measures would include the following:

Avoidance of pregnancy (COE I, LOE C)Immunization against influenza and pneumococcal infection (COE I, LOE C)Psychosocial support (COE IIa, LOE B)Avoidance of excessive physical activity that leads to distressing symptoms, preferring epidural rather than general anesthesia while being taken up for an elective surgery (COE IIa, LOE C).

Supportive therapy

Diuretics: Used to treat fluid retention or when patient has signs of RV failure (COE I, LOE C)Oxygen therapy: Continuous long-term O2 therapy is recommended in PAH patients when arterial blood O2 pressure is consistently <60 mmHg (COE I, LOE C)Anticoagulation therapy: Controversial may be considered (COE IIb, LOE C).Correction of anemia and/or iron status may be considered (COE IIb, LOE C).

Acute vasoreactivity testing

The current consensus definition of a positive response is defined as a fall in mean pulmonary arterial pressure (mPAP) of at least 10 mmHg to a mPAP of 40 mmHg or less with unchanged or increased CO. Patients who meet these criteria may be treated with a CCB agent and should be monitored closely for both safety and efficacy of therapy.

Calcium channel-blocking agents

Patients who meet these criteria may be treated with a CCB agent and should be monitored closely for both safety and efficacy of therapy (COE I, LOE C). Patients with IPAH should be initiated on high-dose CCBs and should be continued on high dose in WHO-FC I or II with marked hemodynamic improvement.

Pulmonary arterial hypertension-specific therapy

Patients with initiation of PAH-specific therapy in WHO-FC III or IV or those without marked hemodynamic improvement even after high doses of CCBs get a COE I, LOE C.

Monotherapy

Prostacyclin receptor agonists: Epoprostenol, treprostinil (intravenous or subcutaneous), iloprost (inhaled), and selexipag (oral)Endothelin receptor antagonists: Bosentan, macitentan (nonselective), and ambrisentan (selective)Phosphodiesterase 5 inhibitors: Sildenafil and tadalafilGuanylate cyclase stimulators: RiociguatMonotherapy with ambrisentan/bosentan/sildenafil in WHO-FCII, IIIMonotherapy with macitentan/tadalafil/riociguat WHO-FC II and III and may be in WHO-FC IVMonotherapy with parenteral prostacyclins is indicated in WHO III and IV.[7],[8],[9],[10],[11],[12],[13],[14],[15],[16]

Combination therapy

Combination therapy may have additive effect or same effect at lower dose of each agent. They can be used as an add-on (sequential) therapy or initiated together, With the best efficacy combination therapy being tadalafil and ambrisentan as shown in AMBITION trial.[12],[13],[14],[15],[16]

Surgical options

Surgical options are indicated in severe symptomatic patients with PAH, despite medical therapy. It includes the following:

Atrial septostomyPotts shuntBilateral lung or heart-lung transplantation (procedure of choice).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Orłowska-Baranowska E, Baranowski R, Hryniewiecki T. Incidence of syncope and cardiac arrest in patients with severe aortic stenosis. Pol Arch Med Wewn 2014;124:306-12.
2Williams L, Frenneaux M. Syncope in hypertrophic cardiomyopathy: Mechanisms and consequences for treatment. Europace 2007;9:817-22.
3Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, et al. Predicting survival in pulmonary arterial hypertension: Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation 2010;122:164-72.
4Beghetti M, Galiè N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 2009;53:733-40.
5Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: A call for action. Eur Heart J 2014;35:691-700.
6Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation 2014;130:1820-30.
7Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: The joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015;46:903-75.
8D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991;115:343-9.
9Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: Updated ACCP evidence-based clinical practice guidelines. Chest 2007;131:1917-28.
10Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A, et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009;30:394-403.
11Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43:5S-12S.
12Rich S. The current treatment of pulmonary arterial hypertension: Time to redefine success. Chest 2006;130:1198-202.
13McGoon MD, Krichman A, Farber HW, Barst RJ, Raskob GE, Liou TG, et al. Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc 2008;83:923-31.
14Krieger N. Overcoming the absence of socioeconomic data in medical records: Validation and application of a census-based methodology. Am J Public Health 1992;82:703-10.
15Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;111:3105-11.
16Lin D, Wei L, Ying Z. Checking the cox model with cumulative sums of Martingale-based residuals. Biometrika 1993;80:557-72.