A Young Lady with Hypotension and Engorged Neck Veins

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A young lady with hypotension and engorged neck veins

Aggarwal A, Sharma V, Agarwal MP, Giri S

Department of Medicine, 25-year-old woman presented to us with history of breathlessness. The lady had noticed University College of A progressively increasing fatigue in the past six months and had a sense of generalized weakness. Medical Sciences, Delhi, Over the past week or so she had been unable to do even routine chores at home and had been India breathless since the past two days. The dyspnoea increased with lying down. She denied any history of fever. CCorrespondence:orrespondence: Vishal Sharma E-mail: docvishalsharma@ She appeared pale, had dry skin and sparse pubic and axillary hair. Her neck veins were engorged. gmail.com She had a pulse rate of 86/min, blood pressure of 76/40 mm Hg. Pulsus paradoxus was absent. Her chest was clear and heart sounds were distant.

What is the Possible Diagnosis?

Received : 04-04-08 Review completed : 26-05-08 The presentation of the case suggests a subacute to chronic process. The history of orthopnea, Accepted : 12-06-08 engorged neck veins and hypotension indicate a cardiac pathology. The features of engorged pubMed ID : 18626175 neck veins, hypotension and muffled heart sounds constitute the classical Becks’ triad of cardiac J Postgrad Med 2008;54:225-7 tamponade.[1]

A chest roentgenogram revealed massive cardiomegaly. age collagen vascular disease can be a possibility. Also tamponade Electrocardiogram revealed low voltage complexes. An can be the presentation of a yet occult malignancy.[1] echocardiography revealed pericardial effusion with features of cardiac tamponade with collapse of the right atrium and Investigations revealed Hb- 9.8 gm/dL, total leucocytes of 9800 ventricle in diastole. An urgent pericardiocentesis was performed with 75% polymorphs and 23% lymphocytes. ESR was 32. Red and around 350 ml fluid removed. The condition of the patient blood cells were normocytic and normochromic. Liver and renal improved. function tests were normal. The serum Na+ was 132, serum K+ was 3.7 and serum Ca2+ was 9 mg/dL. The pericardial fluid of What are the Possible Causes of Cardiac Tamponade in the patient was slightly yellow. Pericardial fluid examination this Patient? revealed proteins of 4.1 g%, sugar 42 mg% and total cells 50/ ml predominantly lymphocytes. No atypical cells or cholesterol The classical Beck’s triad is not usually seen in slow-developing crystals were observed. Gram and AFB staining, culture and effusion. However, the presence of pallor, dry skin and prolonged PCR for mycobacterium were negative. Adenosine deaminase progressive fatigue suggest a long-drawn process. The possibilities levels were 6 IU/ml. to be considered in chronic pericarditis include chronic infections (tuberculosis, fungal), neoplastic (lymphoma, metastasis from The patient said that she was amenorrhoeic since her last breast, lung), uremia, myxedema, collagen vascular diseases childbirth four years back. She had delivered a child at her (systemic lupus erythematosus (SLE), rheumatoid arthritis, home four years back and had needed hospitalization for scleroderma), severe chronic anemia, chylopericardium etc.[1] severe postpartum bleeding. Following this she had a failure of lactation and had not had a pregnancy ever since. She also In this patient in view of the chronic nature of complaints, complained of loss of libido, hair loss, increasing fatigue. tuberculosis is a distinct possibility. The presence of dry hairless skin suggests a possibility of hypothyroidism. However, What is the Diagnosis in this Patient? pericardial effusions secondary to hypothyroidism only rarely result in tamponade.[1,2] Although tamponade is rare in SLE, The history of postpartum bleeding suggests Sheehan’s cases of patients of SLE actually presenting with tamponade syndrome. The classical history of lactational failure followed are known.[3] Even drug-induced SLE has occasionally presented by failure of menstruation virtually clinches the diagnosis of with tamponade as first manifestation.[4] In view of her young Sheehan’s syndrome.[5]

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The manifestations in this are believed to result from a correlation between the amount of pituitary remnants and the postpartum necrosis of pituitary due to postpartum hormonal secretory capacity this does not appear to be true in haemorrhage. This is because of higher blood supply needed this case.[7] by pituitary as a result of physiological hypertrophy during pregnancy. However, evidence suggests a role of autoimmune What are the Cardiac Manifestations of Hypothyroidism response to sequestered antigens released as a result of and are they Different in Primary and Central pituitary necrosis in the causation of Sheehan’s syndrome. Hypothyroidism? This ongoing autoimmune phenomenon explains the late presentation of Sheehan’s in the form of circulatory collapse Cardiac manifestations of hypothyroidism include sinus (hypocortisolism and hypothyroidism) occurring long after bradycardia, diastolic hypertension, narrow pulse pressure, the initial manifestations of acute loss of pituitary function pericardial effusions, coronary artery disease etc. Pericardial (agalactia and amenorrheic). In fact it has been seen that a effusions although common with primary hypothyroidism, higher percentage of patients with Sheehan’s syndrome had are rare in central hypothyroidism.[2] Hypothyroidism-related pituitary antibodies as compared to controls.[6] pericardial effusions are usually clear and straw colored. Occasionally they may be yellow (gold paint) due to cholesterol Sheehan’s syndrome can present with variable manifestations pericarditis.[8] They only rarely cause tamponade even in primary depending on the involvement of the pituitary. The features hypothyroidism. Usually, the hypothyroidism in Sheehan’s may include postpartum lactational failure and amenorrheic, syndrome is less severe than in primary hypothyroidism.[5] This fatigue, weakness, hair loss, fine wrinkles around face, loss of patient presented with features of cardiac tamponade which was libido, polyuria, hypoglycemia and hypotension etc.[5] confirmed on echocardiography.

Her hormonal studies revealed a serum TSH-0.6 (0.35-5.5 Is Cardiac Tamponade Described with Sheehan’s mU/L), serum free T3- 0.8 (2.3-4.2 pg/mL), serum free T4-0.2 Syndrome? (0.9-2.80 ng/dL), serum FSH-4.75 (23-116 IU/L), serum LH-1.83 (15.9-54.0 U/L), serum cortisol (8 AM morning)-1(5-23 ug/dL). Cases of Sheehan’s syndrome presenting as tamponade have These are consistent with the possibility of panhypopituitarism. been reported earlier.[8-10] Cardiac tamponade is rare with The serum TSH can be normal, low or sometimes raised in hypothyroidism due to earlier detection of hypothyroidism. But central hypothyroidism. Adrenocorticotropic hormone is usually in cases presenting late this can be seen. Cardiac tamponade low (not available in our patient). occurs when the amount of fluid in the pericardium is sufficient to restrict the inflow of blood into the ventricles. The amount What are the Causes of Panhypopituitarism? of fluid necessary to cause tamponade varies with the rapidity of development of effusion. In hypothyroidism, since the process The possible causes to be considered in this patient apart from is slow, tamponade is unusual. In such cases tamponade can Sheehan’s include lymphocytic hypophysitis or other infiltrative develop slowly and may mimic heart failure with features of diseases, pituitary adenomas or a parasellar mass, tuberculosis dyspnea, orthopnea, tender hepatomegaly.[1] In Sheehan’s or rarely, empty sella syndrome. Magnetic resonance imaging syndrome the presence of low cortisol may result in low-pressure of the brain using gadolinium contrast revealed a normal tamponade. In low-pressure tamponade, usually occurring in pituitary, sella and parasellar region. Imaging in Sheehan’s hypovolemia, the features can be subtle and easily missed. Here usually reveals an empty sella of normal size although enlarged the jugular venous pressure is usually normal or only slightly or partially atrophied pituitary may be seen depending on the elevated, the arterial pressure is normal and pulsus paradoxus stage of the disease. Rarely, as in the present case, sella can be usually absent.[2,8] normal [Figure 1].[5] Although some authors have suggested What are the Lessons from this Case?

With two-thirds of deliveries occurring at home and the poor state of obstetric and general healthcare, Indian women are at a significant risk of developing Sheehan’s syndrome. Although not much literature is available about the epidemiology of Sheehan’s syndrome, a study in Kashmir valley projected that more than 3% of all parous women ≥20 years might be having Sheehan’s syndrome. Very often these women present late with life-threatening complications as in our case.[11]

This case indicates the need to consider hypothyroidism, even central, as a possible etiology in patients with unexplained pericardial effusion. Since Sheehan’s can present at a variable duration after the last pregnancy it has to be considered even if history of childbirth is remote. Lactational failure is seen in Figure 1: MRI showing normal sella most severe cases of Sheehan’s syndrome. Therefore follow-up

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and the cardiomegaly disappeared [Figure 2]. The patient is still on regular follow-up.

References

1. Braunwald E. Pericardial disease. In: Kasper DL, Braunwald E, Fauci A, Hauser SL, Longo DL, Jameson JL, editors. Harrison’s principles of internal medicine. 16th ed. New York: McGraw Hill; 2005. p.1414-20. 2. Larsen PR, Davies TF. Hypothyroidism and thyroiditis. In: Larsen PR, Kronenberg HM, Melmed S, Polonsky KS, editors. Williams textbook of endocrinology. 10th ed. Philadelphia: Saundres; 2003. p. 423-55. Figure 2: Improvement in cardiomegaly with hormone replacement 3. Abraham GE 3rd, Thorne KD, Moore CK. Tamponade precedes diagnosis of systemic lupus erythematosus. Am J Med Sci of patients of postpartum haemorrhage for development of 2006;331:162-3. 4. Verma SP, Yunis N, Lekos A, Crausman RS. Carbamazepine-induced lactational failure may help in preventing more serious late systemic lupus erythematosus presenting as cardiac tamponade. complications like hypothyroidism and hypocortisolism. Chest 2000;117:597-8. 5. Keleştimur F. Sheehan’s syndrome. Pituitary 2003;6:181-8. The management consists of management of anemia, 6. Goswami R, Kochupillai N, Crock PA, Jaleel A, Gupta N. Pituitay autoimmunity in patients with Sheehan’s syndrome. J Clin Endocrinol hypotension, hypoglycaemia and any other complication. Metab 2002;87:4137-41. Adequate replacement of deficient hormones should be done. 7. Lee HC, Lee EJ, Lee KW, Ahn KJ, Jung TS, Kim DI, et al. Computed With appropriate hormone replacement most patients make tomographic correlation with pituitary function in Sheehan’s a complete recovery over a variable duration. However, the syndrome. Korean J Intern Med 1992;7:48-53. 8. Goswami R, Tandon N, Singh B, Shah P, Ammini AC. Circulatory worsening of tamponade with thyroxine replacement has been collapse in a 30 year old amenorrheic woman. Postgrad Med J seen occasionally and is due to precipitation of cholesterol 1996;72:501-4. crystals leading to cholesterol pericarditis. This emphasizes the 9. Sahin I, Taskapan H, Yildiz R, Kosar F. Cardiac tamponade as initial need for adequate monitoring during therapy.[8] In severe cases presentation of Sheehan’s Syndrome. Int J Cardiol 2004;94:129- 30. and in the presence of fluid depletion treatment should be on 10. Tahir MZ. Cardiac tamponade in hypothyroidism due to Sheehan’s lines of adrenal crisis. It is important to start steroids prior to syndrome. J Pak Med Assoc 1992;42:249-51. thyroxine replacement because thyroxine replacement can lead 11. Zargar AH, Singh B, Laway BA, Masoodi SR, Wani AI, Bashir MI. Epidemiologic aspects of postpartum pituitary hypofunction to adrenal crisis by enhancing the metabolism of glucocorticoids (Sheehan’s syndrome). Fertil Steril 2005;84:523-8. [12] and increasing the metabolic needs of the body. 12. Schneider HJ, Aimaretti G, Kreitschmann-Andermahr I, Stalla GK, Ghigo E. Hypopituitarism. Lancet 2007;369:1461-70. After the initial pericardiocentesis the lady was put on hormone replacement including stepwise uptitrated thyroxine and Source of Support: Nil, Confl ict of Interest: Not declared. hydrocortisone was initiated. The patient improved remarkably

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