Abstract
Primary pulmonary hypertension is a well known entity with characteristic features more common in females presenting commonly with dyspnea. However primary pulmonary hypertension presenting as hoarseness of voice is rare occurring most likely due to compression of left recurrent laryngeal nerve between normal aorta and dilated tense pulmonary artery. Here we are presenting a case of 19 year old boy with primary pulmonary hypertension who presented with hoarseness of voice as predominant symptom.
Keywords: Primary pulmonary hypertension, Hoarseness of voice, Dilated pulmonary artery, Cardiovocal syndrome
Abbreviations: PND, paroxysmal nocturnal dyspnea; LRLNP, left recurrent laryngeal nerve palsy
1. Introduction
Hoarseness of voice is a commonly described entity in patients with severe mitral stenosis. Several cardiopulmonary conditions are associated with left recurrent laryngeal nerve palsy. The syndrome is termed as cardiovocal syndrome or Ortner's syndrome. Here we are describing a patient who presented with hoarseness of voice and was subsequently diagnosed as a case of primary pulmonary hypertension with enlarged pulmonary artery causing left recurrent nerve palsy.
2. Case report
19 year old male presented with hoarseness of voice of one month duration. He denied any history of fever, cough, hemoptysis, palpitations or PND. There was no history of rheumatic fever. There was no history of smoking or vocal cord abuse.
On detailed questioning he also admitted having dyspnea on moderate exertion noticed since one year without significant progression. On examination pulse was 72 per minute and regular. Systemic blood pressure was 130/76 mm of Hg. There was no cyanosis, clubbing, lymphadenopathy, raised jugular venous pressure or pedal edema.
Apex beat was in 5th intercostal space 1 cm lateral to mid clavicular line, tapping in character. Left parasternal heave was noted with palpatory S2 in the left second intercostal space. S1 was normal with accentuated P2 and grade 3/6 systolic murmur at left lower sternal border increasing on inspiration.
Electrocardiogram showed right axis deviation, right ventricular hypertrophy with strain pattern with clockwise rotation of heart. Chest X-ray showed cardiomegaly with enlarged and dilated pulmonary artery and oligemic lung fields.
2D echocardiography showed grossly dilated right atrium, right ventricle and pulmonary artery. On color Doppler there was severe tricuspid regurgitation with severe pulmonary arterial hypertension (100.5 mm Hg) (Fig. 1) and moderate PR with pulmonary artery end diastolic pressure by PR jet 38 mm Hg. Mitral valve apparatus was normal and there was no aortic dilatation.
Fig. 1.
2 D images with color Doppler showing severe TR. (tricuspid regurgitation).
Indirect larynscopy showed paralyzed left vocal cord confirmed with direct fibreoptic laryngoscopy.
CT pulmonary artery (Fig. 2) with CECT thorax confirmed findings of enlarged pulmonary artery and excluded any other intrathoracic lesion.
Fig. 2.
CT pulmonary artery demonstrating enlarged pulmonary artery.
3. Discussion
Norbert Ortner, an Austrian physician first described this syndrome in 1897 implicating hoarseness of voice to the left recurrent laryngeal nerve palsy (LRLNP) in three patients with severe mitral stenosis and postulated that LA dilatation was causing LRLNP by compression against arch of aorta.1
On the basis of the autopsy studies, Fetterrolf and Norris showed that the distance between the aorta and pulmonary artery within the aortic window is only 4 mm and suggested that compression of the nerve between the two structures is responsible for palsy.2 This hypothesis was further strengthened when compression of LRLN was observed by Ari et al between aorta and pulmonary artery near the ligamentum arteriosum in patients with mitral stenosis undergoing mitral commissurotomy.3
As more cases of hoarseness of voice with cardiac disorders were reported, more theories were described such as lymphadenitis and scarring in the aortic window causing nerve fixation,4 pressure from the left bronchus,4 right ventricular hypertrophy,4 pulmonary artery atherosclerosis,4 anatomical position of the ligamentum arteriosum5 or a dilated pulmonary artery.6
It is important to understand anatomy before concluding cause effect relationship.
The left vagus nerve gives rise to the left recurrent lagyngeal nerve at the level of the aortic arch curve. The left recurrent laryngeal nerve then curves around the aorta on the outer side of the ligamentum arteriosum and ascends in the groove between the esophagus and the trachea. The nerve then continues along the trachea-oesophageal groove to supply all the muscles of the left vocal cord except the cricothyroid muscle. This course makes it vulnerable to injury due to pathological conditions in surrounding structures.
Most recently it is suggested that compression of the nerve between the dilated, hypertensive pulmonary artery and the aorta is the constant factor in almost all cardiovascular cases with increase in pulmonary pressure and dilatation of pulmonary artery.4 In all cases where measured, the pulmonary arterial pressure was raised, and the palsy may be relieved by mitral valve surgery if the pulmonary arterial pressure is reduced.7
Primary pulmonary hypertension is a well known entity presenting commonly with dyspnea (80%), fatigue (19%) and syncope (13%). As name suggests cause is unexplained. There have been few case reports of PPH presenting with hoarseness of voice.8,9
This patient with no other etiology with severe pulmonary hypertension and dilated pulmonary artery presented with hoarseness of voice is most likely due to compression of the left recurrent laryngeal nerve between normal aorta and the dilated tense pulmonary artery. Patient was started on diuretics, digoxin, warfarin and calcium channel blocker (amlodepine). Patient had a definite improvement in hoarseness of voice over next 2 months observed in follow up.
4. Conclusion
Hoarseness of voice occurs in cardiac diseases most probably due to compression of left recurrent nerve between aorta and tense and dilated pulmonary artery and can improve with measures decreasing pulmonary pressure.
One should also keep in mind cardiac causes in unexplained hoarseness of voice with left recurrent laryngeal nerve palsy and look for it.
Conflicts of interest
All authors have none to declare.
Footnotes
Work originated at Sir Sunderlal Hospital, Banaras Hindu University, Varanasi.
References
- 1.Ortner N. Recurrenslahmung bei mitral stenose. Wien Klin Wochenschr. 1897;10:753–755. [Google Scholar]
- 2.Fetterolf G., Norris G. The anatomical explanation of paralysis of left recurrent laryngeal nerve found in certain case of mitral stenosis. Am J Med Sci. 1911;141:625–638. [Google Scholar]
- 3.Ari R., Harvey W.P., Hufnagel C.A. Etiology of hoarseness associated with mitral stenosis: improvement following mitral surgery. Am Heart J. 1955:153–160. doi: 10.1016/0002-8703(55)90261-1. [DOI] [PubMed] [Google Scholar]
- 4.Subramaniam Vijayalakshmi, Adarsha Herle T.V., Mohammed Navisha, Thahir Muhammad. Ortner's syndrome: – case series and literature review. Braz J Otorhinolaryngol. 2011;77:559–562. doi: 10.1590/S1808-86942011000500004. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Dolowitz D.A., Lewis C.S. Left vocal cord paralysis associated with cardiac disease. Am J Med. 1948:856–862. doi: 10.1016/0002-9343(48)90482-3. [DOI] [PubMed] [Google Scholar]
- 6.Rosenberg S.A. A study of the etiological basis of primary pulmonary hypertension. Am Heart J. 1964;68:484–489. doi: 10.1016/0002-8703(64)90149-8. [DOI] [PubMed] [Google Scholar]
- 7.Camishion R.C., Gibbon J.H., Jr., Pierucci L., Jr., Iida J. Paralysis of the left recurrent laryngeal nerve secondary to mitral valvular disease. Ann Surg. 1966;163:818–828. doi: 10.1097/00000658-196606000-00002. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Shah K.D., Ayyer K.H., Shah U.K. Hoarseness of voice a presenting manifestation of primary pulmonary hypertension. Indian J Otolaryngol. june 1980;32:35–36. [Google Scholar]
- 9.Wilmshurst P.T., Webb-Peploe M.M., Corker R.J. Left recurrent laryngeal nerve palsy associated with primary pulmonary hypertension and recurrent pulmonary embolism. Br Heart J. 1983:141–143. doi: 10.1136/hrt.49.2.141. [DOI] [PMC free article] [PubMed] [Google Scholar]