Ayerza syndrome (AS) describes the signs of pulmonary (arterial) hypertension [1]. It is mostly diagnosed in adults and has a higher prevalence in females than males. Defined by a mean pulmonary artery pressure of more than or equal to 25 mmHg and a capillary wedge pressure of over 15 mmHg [2], this medical entity can arise from a number of pathologies that may be cardiac or pulmonary in origin, or related to other systemic diseases, drugs, and toxins. Conditions that precipitate AS include chronic obstructive pulmonary disease (COPD), interstitial lung disease and fibrosis, left heart failure, autoimmune diseases and human immunodeficiency virus (HIV) infection. A significant proportion of cases are idiopathic, with no apparent cause, of which 70% have a mutation in the bone morphogenetic protein receptor type 2 (BMPR2) gene [3] [4] and few have in other genes, for example, activin receptor-like kinase type 1 (ACVRL1). Up to 40% of those with no family history of pulmonary hypertension (PH) also carry the gene mutation [5]. PH is progressive and may lead to death, especially if left untreated. Patients typically experience an insidious onset of symptoms that initially occur during exercise, and worsen as the ailment progresses. Consequently, this causes a delay in presentation and diagnosis. Early signs include dyspnea, syncope, dizziness, and cough [6]. Late manifestations are usually due to the underlying or resultant cardiac dysfunction and venous congestion. Among those reported are angina, hyperactive precordium, paroxysmal nocturnal dyspnea, hemoptysis, raised jugular venous pressure, abnormal heart sounds, and edema. Less common complaints include signs of liver failure such as ascites, and digital clubbing, which appear in an advanced stage.

• Fatigue

  N Engl J Med 1941; 225:845-855 MILITARY SYMPOSIUM FATIGUE IN AIRCRAFT PILOTS* Ross A. McFarland, PH.D.\s d\ BOSTON ONE of the most significant problems in civil and military aviation relates to what is popularly known as "pilot fatigue." [nejm.org]

  NEWS.com.au Top Stories In particular, fatigue does not improve, possibly due to co-existing medical problems, such as obstructive sleep apnea or obesity hypoventilation, according to Dr, Greening. [wordnik.com]

  724.8 Fallot's 745.2 falx (see also Hemorrhage, brain) 431 familial eczema-thrombocytopenia 279.12 Fanconi's (anemia) (congenital pancytopenia) 284.09 Fanconi (-de Toni) (-Debré) (cystinosis) 270.0 Farber (-Uzman) (disseminated lipogranulomatosis) 272.8 fatigue [theodora.com]

• Plethora

  Diagnosis and investigation of AS involve a plethora of invasive and non-invasive techniques. These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms. [symptoma.com]

• Virilization

  Morgagni) (syncope with heart block) 426.9 Addisonian 255.41 Adie (-Holmes) (pupil) 379.46 adiposogenital 253.8 adrenal hemorrhage 036.3 meningococcic 036.3 adrenocortical 255.3 adrenogenital (acquired) (congenital) 255.2 feminizing 255.2 iatrogenic 760.79 virilism [theodora.com]

• Difficulty Walking

  People in class three have difficulty walking or climbing stairs, so this class has moderate limits. Physical activity is very limited in class four, as you may have pulmonary hypertension symptoms even at rest. [brighthub.com]

• Dyspnea

  Among those reported are angina, hyperactive precordium, paroxysmal nocturnal dyspnea, hemoptysis, raised jugular venous pressure, abnormal heart sounds, and edema. [symptoma.com]

  Ayerza's disease Ayer·za's disease ə-'yər-zəz- n a complex of symptoms marked esp. by cyanosis, dyspnea, polycythemia, and sclerosis of the pulmonary artery Ayerza ä-'yer-sä Abel (1861-1918) Argentinean physician. [medicine.academic.ru]

  Condition characterized by dyspnea, slowly developing asthma, bronchitis, and chronic cyanosis in association with polycythemia. Polycythemia usually results from chronic pulmonary insufficiency and sclerosis of the pulmonary vascular bed. [medigoo.com]

  Abstract In 1919 Aldred Scott Warthin 1 published a clinical report and autopsy protocol entitled "A Case of Ayerza's Disease: Chronic Cyanosis, Dyspnea, and Erythremia, Associated with Syphilitic Arteriosclerosis of the Pulmonary Artery." [annals.org]

  a stevedore, white, aged 48, was first seen in October, 1924, complaining of a cough productive of a small amount of mucus, a feeling of constriction across the upper part of the chest and dyspnea, which had developed over a period of five years. [jamanetwork.com]

• Clubbed Finger

  Dyspnoea was severe at rest and there was central cyanosis, clubbed fingers and tachypnea. In the respiratory examination, wet crackles and wheezing were abundant in both lungs. [err.ersjournals.com]

• Hepatomegaly

  In the cardiovascular examination there was jugular venous distension, hepatomegaly, hepatojugular reflux, ascites and oedema of lower limbs. [err.ersjournals.com]

  […] artery 093.89 heart 093.89 block 093.89 decompensation 093.89 disease 093.89 failure 093.89 valve (see also Syphilis, endocarditis) 093.20 hemianesthesia 094.89 hemianopsia 095.8 hemiparesis 094.89 hemiplegia 094.89 hepatic artery 093.89 hepatitis 095.3 hepatomegaly [theodora.com]

• Cyanosis

  A·yer·za syn·drome ( ah-yār'shah ), sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary arteriosclerosis or primary pulmonary [medical-dictionary.thefreedictionary.com]

  Medical Term: Pronunciation: ah-yār′shah Definition: sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary arteriosclerosis or [rxmed.info]

  It is associated with severe cyanosis. Also called cardiopathia nigra. Read Also: Ayesha Aisha. Historical Examples Gone was the corpse of Leo, and gone too was Ayesha the imperial, the divine. Ayesha H. [definithing.com]

  Ayerza syndrome: Description, causes and risk factors Ayerza syndrome - sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary [medigoo.com]

• Irregular Heart Rhythm

  The EKG gives doctors a record of your heartbeat, which helps identify irregular heart rhythms. Classification Pulmonary hypertension does limit physical activity in some cases. [brighthub.com]

• Difficulty Walking and Climbing Stairs

  People in class three have difficulty walking or climbing stairs, so this class has moderate limits. Physical activity is very limited in class four, as you may have pulmonary hypertension symptoms even at rest. [brighthub.com]

• Torticollis

  […] exudativum) 695.1 Stewart-Morel syndrome (hyperostosis frontalis interna) 733.3 Sticker's disease (erythema infectiosum) 057.0 Stickler syndrome 759.89 Sticky eye 372.03 Stieda's disease (calcification, knee joint) 726.62 Stiff back 724.8 neck (see also Torticollis [theodora.com]

• Flushing

  […] hemorrhagic pancreatitis) 577.0 Fitz-Hugh and Curtis 098.86 due to Chlamydia trachomatis 099.56 Neisseria gonorrhoeae (gonococcal peritonitis) 098.86 Flajani (-Basedow) (exophthalmic goiter) 242.0 floppy infant 781.99 iris 364.81 valve (mitral) 424.0 flush [theodora.com]

• Somnolence

  […] brain) 013.2 ulcer, bladder 596.8 Somatization reaction, somatic reaction (see also Disorder, psychosomatic) 306.9 disorder 300.81 Somatoform disorder 300.82 atypical 300.82 severe 300.81 undifferentiated 300.82 Somnambulism 307.46 hysterical 300.13 Somnolence [theodora.com]

Diagnosis and investigation of AS involve a plethora of invasive and non-invasive techniques. These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms [7]. The diagnostic test for AS is right heart catheterization, as it allows direct measurement of the pulmonary arterial pressure (which will be above 25 mmHg) and thus confirms the condition. Pulmonary capillary wedge pressure may be within the normal range.

Laboratory examination includes full blood count, assessment of liver, thyroid and renal function, immunological assays, arterial blood gas analysis (ABGA), and human immunodeficiency virus (HIV) testing [8]. Cardiac biomarkers such as brain natriuretic peptide (BNP) may be raised, indicating heart abnormalities [9].
Imaging may involve:

• Electrocardiogram (ECG): Shows signs of ventricular hypertrophy.
• Echocardiogram.
• Spirometry, ventilation-perfusion (V/Q) scanning, and pulmonary angiography to measure lung function.
• Administration of vasodilators to provoke a dilation of the pulmonary vessels, such as nitric oxide and adenosine, as well as other tests for staging and treatment planning [10].
• Chest X-ray: Shows increased lung perfusion as well as enlarged pulmonary vessels.
• Computerized tomography: Useful in chronic thromboembolic pulmonary arterial hypertension.
• Magnetic resonance imaging (MRI).

• Hypercapnia

  Blood tests reveal a hyperglobulia and a polyglobulia, an anoxemia and often a hypercapnia (see). On an ECG — signs of the expressed hypertrophy and an overload of the right departments of heart. [bigmed.info]

• Ventricular Hypertrophy

  Cardiac biomarkers such as brain natriuretic peptide (BNP) may be raised, indicating heart abnormalities.Imaging may involve: Electrocardiogram (ECG): Shows signs of ventricular hypertrophy. Echocardiogram. [symptoma.com]

  Long-term results of dualchamber (DDD) pacing in obstructive hypertrophic cardiomyopathy: evidence for progressive symptomatic and hemodynamic improvement and reduction of left ventricular hypertrophy. Circulation 1994;90: 2731–2741. 242. [cardiologieladomiciliu.ro]

1. Mazzei JA, Mazzei ME. A tribute: Abel Ayerza and pulmonary hypertension. Eur Respir Rev. 2011;20(122):220–221.
2. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(1 Suppl):35S–62S.
3. Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000;67(3):737–744.
4. Cogan JD, Pauciulo MW, Batchman AP, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174(5):590–598.
5. Machado RD, Aldred MA, James V, et al. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006;27(2):121–132.
6. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336(2):111–117.
7. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):40S–47S.
8. Raymond R, Hinderliter A, Willis P, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214–1219.
9. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003;108(7):844–848.
10. Enright PL, McBurnie MA, Bittner V, et al. The 6-minute walk test: A quick measure of functional status in elderly adults. Chest. 2003;123(2):387-398.