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 Table of Contents  
Year : 2016  |  Volume : 2  |  Issue : 1  |  Page : 43-48

A Case of pulmonary artery hypertension

Department of Cardiology, AIIMS, New Delhi, India

Date of Web Publication26-May-2016

Correspondence Address:
Anand Palakshachar
Department of Cardiology, AIIMS, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2395-5414.182996

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A patient presented with dyspnea, chest pain, and hemoptysis. Clinical findings revealed evidence of severe pulmonary hypertension and cyanosis with clubbing and a wide split second sound. Investigations including cardiac catheterization and oxygen study were performed, and the patient was referred for surgical repair. This article discusses various aspects of history, clinical examination, and cardiac catheterization that were utilized to decide the management.

Keywords: Atrial septal defect, bedside case discussion, pulmonary artery hypertension

How to cite this article:
Palakshachar A, Bansal R, Gupta SK, Ramakrishnan S. A Case of pulmonary artery hypertension. J Pract Cardiovasc Sci 2016;2:43-8

How to cite this URL:
Palakshachar A, Bansal R, Gupta SK, Ramakrishnan S. A Case of pulmonary artery hypertension. J Pract Cardiovasc Sci [serial online] 2016 [cited 2022 Dec 5];2:43-8. Available from: https://www.j-pcs.org/text.asp?2016/2/1/43/182996

  Clinical Presentation Top

A 24-year-old female, a homemaker from Bihar, presented to the outpatient department with:

  • Dyspnea on exertion for 1 year
  • Chest pain for 1 year
  • One episode of hemoptysis
  • Palpitation for 7 days.

The patient was apparently well till about 1 year ago when she developed pain in the left side of the chest. This was nonradiating, more on exertion and used to get relieved by rest in 2–5 min. There was no history of ingestion of nitrates to relieve pain. The pain intensity kept increasing with the passage of time and it started occurring with less exertion. There was no associated fever, cough, sputum, and hemoptysis. The onset of the symptoms was 20 days after her first childbirth.

Along with this, she developed shortness of breath, which was not very severe (New York Heart Association Class II) and was not associated with orthopnea and paroxysmal nocturnal dyspnea. There was no worsening of dyspnea during the next 1 year. She also had one episode of hemoptysis (about 10 ml of blood) 6 months ago. This settled spontaneously with medications over 2 days.

She also gave a history of palpitations on exertion for the past 7 days. These were present only on exertion and relieved by rest. They gave a pounding sensation on the chest.

There was no history of presyncope, syncope, or seizures. There was no history of arthralgia or arthritis or taking monthly injections. There was no history of chronic cough, expectoration, and history of tuberculosis in the family. There was no history of noticing bluishness of nails or tongue at any time. No history of recurrent chest infections in her childhood has been reported.

Her menstrual history was normal and her only child was normal. Her pregnancy was normal but her symptoms started 20 days after her first childbirth. The child was born of uncomplicated, normal vaginal delivery in a hospital. There is no history of excess blood loss during the procedure. There was no history of prolonged fever or prolonged per vaginal discharge after delivery.

There was no history of similar illness in the family.

  Summary Top

A 24-year-old lady has presented with 1 year of nonprogressive dyspnea with exertional chest pain and palpitation, along with one episode of hemoptysis and palpitations. Although her pregnancy and early postpartum period was uneventful, she became symptomatic after 20 days of childbirth.

  Discussion Top

This is a short history of nonprogressive dyspnea, chest pain, and hemoptysis in a female with symptoms starting after childbirth.

The differential diagnosis includes the following.


Dilated cardiomyopathy is a possibility in view of the history of recent childbirth. The dyspnea is nonprogressive but could happen if the left ventricular (LV) dysfunction was mild and improved subsequently. There is no pointer in the history to suggest restrictive cardiomyopathy. The possibility of hypertrophic cardiomyopathy cannot be ruled out although occurrence of symptoms soon after childbirth is odd for this diagnosis.

Stenotic valvular lesions

Aortic stenosis related to bicuspid aortic valve is possible in view of chest pain and dyspnea. However, uneventful pregnancy is again unlikely in a patient with symptomatic severe aortic stenosis.

Rheumatic heart disease

There are no pointers in the history to suggest rheumatic heart disease. Nevertheless, considering high prevalence in India, this possibility cannot be ruled out especially in view of history of dyspnea and one episode of hemoptysis. There is no history of penicillin prophylaxis.

Ruptured sinus of valsalva (RSOV): Chest pain in a young person can suggest RSOV but relatively static course makes it less likely.

Pulmonary artery hypertension (PAH): Taking hemoptysis as a clue, Eisenmenger's syndrome or severe PAH of any etiology is a possibility. Dyspnea is also explained.


What are the cardiovascular problems which can have an onset in the peripartum period?

Stenotic valvular heart diseases such as mitral stenosis (MS) and aortic stenosis because of the rapid increase in volume status lead to decompensation of hemodynamics, peripartum cardiomyopathy (PPCM), preeclampsia and eclampsia, deep vein thrombosis (DVT), and pulmonary embolism (PE).

Which are the cardiovascular problems where the triad of dyspnea, chest pain, and hemoptysis can be there?

  • PE
  • PAH of any origin
  • MS
  • Cyanotic congenital heart disease with decreased pulmonary flow.

What is the natural course of PPCM?

Patients with PPCM have a better prognosis than other forms of cardiomyopathy. A study of natural course of disease in 27 patients [1] over 21 years, showed 14 patients were free of heart failure symptoms by 6 months and cardiac size normal on chest X-ray. Remaining 13 patients did not show regression of cardiomegaly at 6 months. Ten patients were dead within 8 years due to heart failure. Nearly 50–60% recovery has been shown.[2] Subsequent pregnancies may result in recurrence of heart failure, more in patients who have residual LV dysfunction and can be fatal in patients who remained symptomatic. Studies have shown recovery is greatest in patients with ejection fraction <30% and impaired when LV dimension was >56 mm.

What is the natural history of Eisenmenger syndrome? When does it present in adults? What is the usual mode of presentation? What are the usual symptoms? What ages do different levels of shunts present?

Life expectancy is reduced by 20 years. Survival rates at 1, 5, 10, 15, and 25 years are 97, 87, 80, 77, and 42%, respectively. Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood. Paul Wood described the average age of presentation of patent ductus arteriosus (PDA), ventricular septal defect (VSD), and atrial septal defect (ASD) with Eisenmenger reaction to be 19, 22, and 35 years, respectively. Patients usually present with effort intolerance over a few years, angina on exertion, congestive heart failure, hemoptysis, syncope or presyncopal attacks, symptoms of organ ischemia like stroke due to paradoxical embolization, DVT, and PE due to erythrocytosis. Death occurs suddenly in one-third of patients, massive hemoptysis and right heart failure in remaining majority of the patients. Death due to infective endocarditis, cerebral abscess, and pregnancy is also described.

How common is hemoptysis in MS, Eisenmenger, and RSOV?

In a carefully analyzed series of 107 cases of rheumatic heart disease, Hodes [3] found history of hemoptysis in 48% in a paper published from Ethiopia in 1992. With development of effective treatment, hemoptysis is rare in patients with pure MS presently. Hemoptysis may occur by rupture of thin-walled, dilated bronchial veins, usually as a consequence of a sudden rise in left atrial pressure, or the pink frothy sputum characteristic of acute pulmonary edema with rupture of alveolar capillaries also may develop in these patients. Hemoptysis also may be caused by pulmonary infarction or complication of anticoagulation therapy.

One-third of patients with Eisenmenger syndrome have hemoptysis as a presenting feature. It can be fatal due to massive hemoptysis from pulmonary infarction. Other causes are rupture of aortopulmonary collaterals (APCs), coagulopathy, and pulmonary arteriolar rupture.

RSOV leading to acute pulmonary edema can cause pink frothy sputum.

  Examination Top

On examination, the patient was sitting comfortably and was well oriented to time, place, and person.

She had a pulse rate of 94/min. The pulse was a good volume pulse, regular in rate, and symmetric in both upper and lower limbs. Blood pressure in right upper limb in supine position was 114/70 mmHg and respiratory rate was 16/min.

There was Grade II clubbing and mild cyanosis which was uniform in all four limbs.

Cardiovascular examination revealed a Grade II parasternal heave, a palpable second sound, and a normal tapping apex. Auscultation finding included a normal first sound, a second sound which showed a wide fixed split with a loud P2. There was no audible murmur.

Respiratory system was normal.


What is the cause of clubbing in this case?

Central cyanosis due to right to left shunt across the ASD.

What is the grading of clubbing?

  • Grade 1 - Fluctuation and softening of nail bed
  • Grade 2 - Loss of nail bed angle demonstrated by Schamroth sign
  • Grade 3 - Parrot beaking or drumstick appearance
  • Grade 4 - Proximal joint involvement (secondary hypertrophic osteoarthritis).

How is the parasternal heave different in tetralogy of fallot (TOF) and Eisenmenger syndrome due to the different shunts?

Parasternal heave is usually absent in TOF. Right ventricle (RV) is decompressed by a large mal-aligned VSD and overriding of aorta, the blood immediately directed to aorta and there is concentric hypertrophy of RV without enlargement. However, in Eisenmenger physiology, in ASD and PDA, the PA pressure overload can cause an RV parasternal heave.

What are the causes of a wide-fixed split second sound?

ASD, right bundle branch block (RBBB), and RV systolic dysfunction.

How is the second sound used to distinguish the various types of Eisenmenger syndrome?

Second sound is usually single or closely split in Eisenmenger syndrome due to VSD whereas about half of PDA and nearly 90% of ASD will have wide splitting of second sound. Split will widen with inspiration in a normal manner in cases with PDA.

Diagnosis based on history and examination:

  • Severe PAH
  • ASD (ostium secundum)
  • Right to left
  • Normal sinus rhythm
  • No congestive heart failure (CHF).

  Discussion Top

What is the usual age of manifestation with Eisenmenger syndrome?

As shown in Paul Wood's data,[4] 79% of PDA present in infancy and 17% in adulthood; in VSD, 83% in infancy, 15% in childhood and 2% in adulthood; and in ASD, 8% in infancy and 92% in adulthood.

What is the cause of hemoptysis in Eisenmenger syndrome?

Hemoptysis can be caused by pulmonary artery thrombosis, pulmonary artery dissection, PE, rupture of APCs, and hemorrhage due thin-walled arterioles, infection, or bleeding diathesis. In Wood's series, it never occurred before 24 years and the frequency was almost 100% by 40 years.

What features help distinguish the various types of intracardiac shunts?

In a PDA with flow reversal, effort intolerance is slight, the patient is often acyanotic, differential cyanosis may be pathognomonic, and the second sound has a normal split which widens in inspiration. Chest X-ray may reveal a rounded shadow around the aortic knuckle or calcification. In a VSD with PAH, breathlessness, cyanosis and clubbing date from infancy or childhood. History of squatting may be there. Second sound is single. There is no pan systolic murmur. A transposition of the great arteries (TGA) or corrected TGA with VSD is similar to Eisenmenger complex except that on a chest X-ray the pedicle is narrow. In ASD, symptoms begin late unless it is ostium primum in location as seen in patients with atrioventricular septal defect. Second sound is wide split and does not change with respiration. A large right atria and RV and tense pulmonary artery are also suggestive.

  Investigation Top

ECG: It includes normal sinus rhythm, RV hypertrophy, no RBBB, no LV hypertrophy [Figure 1].
Figure 1: Electrocardiography.

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Chest X-ray: No cardiomegaly, prominent pulmonary artery segment, no lung plethora [Figure 2].
Figure 2: Chest X-ray.

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Peripheral lung fields show low flow.

[Figure 3] and [Figure 4] show ASD (ostium secundum with bidirectional flow).
Figure 3: Atrial septal defect shown.

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Figure 4: Flow across atrial septal defect.

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[Figure 5] and [Figure 6] show echocardiograms with mild tricuspid regurgitation, with severe pulmonary hypertension.
Figure 5: Tricuspid regurgitation.

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Figure 6: Doppler at tricuspid valve.

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Echocardiogram showing situs solitus, atrioventricular and ventriculo-arterial concordance, a large ASD (about 27 mm) with bidirectional flow, with moderate tricuspid regurgitation with severe PAH (predicted PA pressure = RA + 100 mmHg). LV is normal, and other valves are normal. No other shunt.

  Catheterization Top

The catheterization [Figure 7] revealed severe PAH (PA pressure equal to systemic pressure), the pulmonary vascular resistance index (PVRI) was 13.7 wood units (WU), and the PVRI/systemic vascular resistance index (SVRI) ratio was 0.5. The Qp/Qs was 2.7. With inhaled oxygen (the calculations included dissolved oxygen), the PA systolic pressure did not change, the Qp/Qs increased to 3.7, and the PVRI/SVRI ratio became 0.3, and the final PVRI was 4.1.
Figure 7: Catheterization data.

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Diagnosis based on investigations:

  • Severe PAH (vasoreactive)
  • ASD (ostium secundum)
  • Right to left shunt
  • Normal Sinus rhythm
  • No CHF.

The plan decided for this patient was that since the vasoreactivity study had shown the PVRI falling to 4.1 (<6 or <4 recommended in various guidelines), the PVRI/SVRI ratio fell to 0.3 (below 0.3 is safe), but there was no fall in pulmonary artery pressure, and this would result in a borderline case for surgery. Therefore the patient was referred for a fenestrated closure of the ASD.

Examiners What is the way to interpret vasoreactivity studies?

In patients with intracardiac shunts, a decrease in PVRI below 4 WU·m 2 and / or in the ratio of pulmonary: Systemic vascular resistance (PVRI:SVRI) below 0.3 indicates that shunt closure may be performed safely. If the PVRI is between 4 and 8 WU·m 2, temporary balloon occlusion of the shunt, if possible, may provide additional useful information. In some centers, a decrease in PVRI to <6–8 WU·m 2 may be used. The decision to close a shunt is made using all the available information and is not solely dependent on the hemodynamics obtained at cardiac catheterization. Different criteria may be used, especially in older patients, to decide whether ASDs ought to be closed because a number of factors affect atrial-level shunting and streaming that may be unrelated to pulmonary vascular resistance.[5]

Is anticoagulation advised in Eisenmenger syndrome?[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24]

Systemic anticoagulation in patients with Eisenmenger syndrome is controversial as patients suffer from hemostatic abnormalities and also are at increased risk of fatal and life-threatening bleeding complications, particularly significant hemoptysis. There is also a deficiency of coagulation factors and platelet malfunction. As of now, systemic anticoagulation and the use of platelet aggregation inhibitors is not recommended.

Is oxygen useful?[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24]

The routine use of supplemental oxygen at home is not recommended but can be used. The trials have not shown long-term benefit.

What about other drugs?[25],[26],[27],[28],[29],[30],[31],[32]

Other beneficial drugs include the following: based on the BREATHE-5 study, bosentan seems to be safe and effective in Eisenmenger syndrome. The experience with sitaxsentan specifically in Eisenmenger patients remains limited. There are no data on ambrisentan in patients with ES. Preliminary results have demonstrated that sildenafil is safe in patients with severe pulmonary hypertension related to the ES. Tadalafil is another phosphodiesterase type-5 inhibitor reported to benefit in patients with ES.

What is the surgical option?[33],[34]

Heart-lung transplantation and single or bilateral, lung transplantation, with or without repair of relatively simple congenital cardiovascular anomalies, are the options for patients who cannot be managed medically.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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Hodes RM. Hemoptysis in rheumatic heart disease. Trop Geogr Med 1992;44:328-30.  Back to cited text no. 3
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Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006;114:48-54.  Back to cited text no. 24
Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol 2004;94:261-3.  Back to cited text no. 25
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Fernandes SM, Newburger JW, Lang P, Pearson DD, Feinstein JA, Gauvreau K, et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol 2003;91:632-5.  Back to cited text no. 27
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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