Journal of Rare Cardiovascular Diseases

A 48-year-old woman was admitted to our centre with symptoms of right heart failure. Pulmonary arterial hypertension (PAH) was diagnosed. The patient’s general condition deteriorated despiteinitiation of sequential PAH specific therapy and hemodynamic support. Coexisting fungal mural endocarditis led to devastating consequences. Infection presented in a deceptive manner. The patient was afebrile, inflammatory markers were low, blood cultures were negative for bacteria, echocardiography revealed only an increased trabeculation of the right ventricle.

The authors discuss management of decompensated right ventricular failure secondary to chronic pulmonary hypertension. The literature on fungal mural endocarditis is also reviewed. JRCD2013; 1 (2): 33–38

Key words: progressive idiopathic pulmonary arterial hypertension, right ventricular failure, fungal mural endocarditis, sibutramine

progressive idiopathic pulmonary arterial hypertension; right ventricular failure; fungal mural endocarditis; sibutramine

McLaughlin VV, Suissa S. Prognosis of pulmonary arterial hypertension: The power of clinical registries of rare diseases. Circulation 2010; 122: 106–108

Peacock AJ, Murphy NF, McMurray JJV, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30: 104–109.

Simonneau G, Robbins I, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–S54.

Gaine SP, Rubin LJ, Kmetzo JJ, et al. Recreational use of aminorex and pulmonary hypertension. Chest 2000; 118: 1496–1497.

Guven A, Koksal N, Cetinkaya A, et al. Effects of the sibutramine therapy on pulmonary artery pressure in obese patients. Diabetes Obes Metab 2004; 6: 50–55.

Possible Meridia side effects / adverse reactions in 53 year old female http://www.druglib.com/adverse-reactions_side-effects/meridia/seriousn

Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009; 30: 2493–2537.

Aklog L, Williams CS, Byrne JG, et al. Acute pulmonary embolectomy: a contemporary approach. Circulation 2002; 105: 1416–1419.

Come PC, Kim D, Parker JA, et al. Early reversal of right ventricular dysfunction in patients with acute pulmonary embolism after treatment with intravenous tissue plasminogen activator. J Am Coll Cardiol. 1987; 10: 971–978.

Lang IM, Klepetko W. Chronic thromboembolic pulmonary hypertension: an updated review. Curr Opin Cardiol. 2008; 23: 555–559.

Via G, Braschi A. Pathophysiology of severe pulmonary hypertension in the critically ill patient. Minerva Anestesiol. 2004; 70: 233–237.

Jacobs AK, Leopold JA, Bates E, et al. Cardiogenic shock caused by right ventricular infarction: a report from the SHOCK registry. J Am Coll Cardiol. 20 03; 41: 1273 –1279.

Delcroix M, Naeije R. Optimising the management of pulmonary arterial hypertension patients: emergency treatments. Eur Respir Rev. 2010; 19: 204–211.

Ukkonen H, Saraste M, Akkila J, et al. Myocardial efficiency during levosimendan infusion in congestive heart failure. Source Clin Pharmacol Ther. 2000; 68: 522–531.

Goldstein JA. Pathophysiology and management of right heart ischemia. J Am Coll Cardiol. 2002; 40: 841–853.

Lualdi JC, Goldhaber SZ. Right ventricular dysfunction after acute pulmonary embolism: pathophysiologic factors, detection, and therapeutic implications. Am Heart J. 1995; 130: 1276–1282.

Kerbaul F, Rondelet B, Motte S, et al. Effects of norepinephrine and dobutamine on pressure load-induced right ventricular failure. Crit Care Med. 2004; 32: 1035–1040.

Kwak YL, Lee CS, Park YH, et al. The effect of phenylephrine and norepinephrine in patients with chronic pulmonary hypertension. Anaesthesia. 2002; 57: 9–14.

Tisdale JE, Patel RV, Webb CR, et al. Proarrhythmic effects of intravenous vasopressors. Ann Pharmacother. 1995; 29: 269–281.

Barrett LK, Singer M, Clapp LH. Vasopressin: mechanisms of action on the vasculature in health and in septic shock. Crit Care Med. 2007; 35: 33–40.

Rich S, Seidlitz M, Dodin E, et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787–792.

Brent BN, Berger HJ, Matthay RA, et al. Contrasting acute effects of vasodilators (nitroglycerin, nitroprusside, and hydralazine) on right ventricular performance in patients with chronic obstructive pulmonary disease and

pulmonary hypertension: a combined radionuclide-hemodynamic study. Am J Cardiol. 1983; 51: 1682–1689.

Cockrill BA, Kacmarek RM, Fifer MA, et al. Comparison of the effects of nitric oxide, nitroprusside, and nifedipine on hemodynamics and right ventricular contractility in patients with chronic pulmonary hypertension. Chest 2001; 119: 128–136.

Tsapenko M, Tsapenko A, Comfere T, et al. Bhorade S, Christenson J, O’Connor M, et al. Response to inhaled nitric oxide in patients with acute right heart syndrome. Am J Respir Crit Care Med. 1999; 159: 571–579.

Troncy E, Francoeur M, Blaise G. Inhaled nitric oxide: clinical applications, indications, and toxicology. Can J Anaesth. 1997; 44: 973–988.

Hoeper MM, Olschewski H, Ghofrani HA, et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. German PPH study group J Am Coll Cardiol. 2000; 35: 176–182.

Ocal A, Kiris I, Erdinc M, et al. Efficiency of prostacyclin in the treatment of protamine-mediated right ventricular failure and acute pulmonary hypertension. Tohoku J Exp Med. 2005; 207: 51–58.

Trachte AL, Lobato EB, Urdaneta F, et al. Oral sildenafil reduces pulmonary hypertension after cardiac surgery. Ann Thorac Surg. 2005; 79: 194–197.

Lobato EB, Beaver T, Muehlschlegel J, et al. Treatment with phosphodiesterase inhibitors type III and V: milrinone and sildenafil is an effective combination during thromboxane-induced acute pulmonary hypertension. Br J

Anaesth. 2006; 96: 317–322.

Punnoose L, Burkhoff D, Rich S, Horn EM. Right ventricular assist device in end-stage pulmonary arterial hypertension: insights from a computational model of the cardiovascular system. Prog Cardiovasc Dis. 2012; 55(2): 234–243.

Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med. 2001; 22: 547–560.

Vieillard-Baron A, Loubieres Y, Schmitt JM, et al. Cyclic changes in right ventricular output impedance during mechanical ventilation. J Appl Physiol. 1999; 87: 1644 –1650.

Roberts DH, Lepore JJ, Maroo A, et al. Oxygen therapy improves cardiac index and pulmonary vascular resistance in patients with pulmonary hypertension. Chest 2001; 120: 1547–1555.

Pierrotti LC, Baddour LM. Fungal endocarditis, 1995–2000. Chest 2002; 122: 302–310.

Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): ESC/ESCMID/ISC. Eur Heart J. 2009; 30: 2369–2413.

Nguyen, MH, Nguyen, ML, Yu, VL, et al. Candida prosthetic valve endocarditis: prospective study of six cases and review of the literature. Clin Infect Dis 1996; 22: 262–267.

Kearney RA, Eisen HJ, Wolf JE. Nonvalvular infections of the cardiovascular system. Ann Intern Med 1994; 121: 219–230.

Ak K, Adademir T, Isbir S, Arsan S. Right ventricular mural endocarditis presenting as an isolated apical mass in a non-addict patient with congenital deafness and aphasia. Interact Cardiovasc Thorac Surg. 2009; 8: 498–500.

Mullen P, Jude C, Borkon M, et al. Aspergillus mural endocarditis. Clinical and echocardiographic diagnosis. Chest 1986; 90: 451–452.

Matthews JW, McLaughlin W. Acute Right Ventricular Failure in the Setting of Acute Pulmonary Embolism or Chronic Pulmonary Hypertension: A Detailed Review of the Pathophysiology, Diagnosis, and Management. Curr Cardiol Rev. 2008; 4: 49–59.