An adult cystic fibrosis patient presenting with persistent dyspnea: a case report

Title:

An adult cystic fibrosis patient presenting with persistent dyspnea: case report

Author/s

Gary M Onady and Catherine L Farinet [1]

Abstract

Background
Persistent dyspnea is a common finding in the cystic fibrosis patient that typically leads to further work up of an alternative pulmonary etiology. Adult cystic fibrosis patients; however, are growing in numbers and they are living into the ages in which coronary artery disease becomes prevalent. Coronary disease should be included in the consideration of diagnostic possibilities.

Case presentation
A 52-year-old white male with cystic fibrosis was evaluated for exertional dyspnea associated with vague chest discomfort. Diagnostic testing revealed normal white blood cell, hemoglobin and platelet count, basic metabolic panel, fasting lipid profile, HbA1c, with chest radiograph confirming chronic cystic findings unchanged from prior radiographs and an electrocardiogram that revealed sinus rhythm with left anterior fascicular block. Stress thallium testing demonstrated a reversible anteroseptal perfusion defect with a 55% left ventricular ejection fraction. Heart catheterization found a 99% occlusion of the left anterior descending artery extending into the two diagonal branches, with 100% obstruction of the left anterior descending artery at the trifurcation and 70% lesion affecting the first posterior lateral branch of the circumflex artery.

Conclusion
This case report represents the first description in the medical literature of a cystic fibrosis patient diagnosed with symptomatic coronary artery disease. Applying a standard clinical practice guide proved useful toward evaluating a differential diagnosis for a cystic fibrosis patient presenting with dyspnea and chest discomfort.

Patient Characteristics

  • 52 y/o white male
  • Cystic Fibrosis
  • gastroesophageal reflux, Barrett's esophagus, azoospermia, and pancreatic insufficiency
  • Medications include albuterol, ipratropium, fluticasone, dornase alpha, salmeterol, omeprazole, pancreatin and multivitamin

Examination

  • Subjective: Pt has persistent exertional dyspnea associated with vague chest discomfortand a cough with scant sputum production
  • Objective: Physical Examination Tests and Measures:  oxygen saturation of 91%
    -Normal temperature
    -Normal vital signs
    -Weight: 77.1 kg
    -Cardiovascular exam: normal
    -Lungs: coarse breath sounds bilaterally with scattered rales throughout the lung fields
    -Abdomen: normal
    -Clubbing of extremities
    -No peripheral edema
    -(FEV1) had decreased from 50% to 36%
    -Chest radiograph resembled baseline findings with no obvious infiltrate or pneumothroax. Chronic cystic changes but no obvious consolidation
    -Normal white blood cell, hemoglobin and platelet count
    -Electrolytes, albumin, protein and glucose were normal with a 5.1% HbA1c
    -Total cholesterol was 139 mg/dl, LDL 80 mg/dl, HDL 30 mg/dl and triglycerides at 76 mg/dl
    -Electrocardiogram revealed sinus rhythm with a left anterior fascicular block and normal ST findings

Clinical Impression

The patient's complaints of dyspnea do not seem to match up with the examination findings and therefore further evaluation and examination may be necessary to determine the cause of his symptoms.

Summarization of Examination Findings

Most exam findings were normal including white blood cell count which might lead to considering causes other than infection. There were no significant findings of consolidation in the chest radiographs but there was a left anterior fascicular block noted on the electrocardiogram which may be a reason to consider the possibility that the patient's heart could be causing the dyspnea rather than his lungs.

Intervention

  • month-long course of azithromycin, aztreonam and inhaled tobramycin for suspected pulmonary exacerbation of acute super-infection in the setting of underlying chronic CF lung disease
  • Rationale for Progression: One month later, follow-up revealed no improvement in dyspnea and no change in the scant sputum production, despite full adherence to the antibiotic regimen and airway clearance techniques. Exertional discomfort located along the sternum and left anterior chest associated with dyspnea without radiation or pressure sensation was further described but was not associated with palpitations, diaphoresis or nausea. This discomfort improved with rest and was not associated with meals. He did report the need to sleep upright in a recliner, but denied paroxysmal nocturnal dyspnea. Further testing was completed and the patient was evaluated for possible coronary artery bypass graft; however, because of his current pulmonary status, angioplasty was elected with successful stenting of the left anterior descending artery.

Outcomes

One month later, follow-up revealed no improvement in dyspnea and no change in the scant sputum production, despite full adherence to the antibiotic regimen and airway clearance techniques.
Stress thallium testing: positive for moderate anteroseptal area of reversible perfusion with a left ventricular ejection fraction of 55%. Subsequent heart catheterization revealed 99% occlusion of the left anterior descending artery with extension into the two diagonal branches, 100% obstruction of the left anterior descending artery at the trifurcation and 70% lesion affecting the first posterior lateral branch of the circumflex artery. The patient was evaluated for possible coronary artery bypass graft; however, because of his current pulmonary status, angioplasty was elected with successful stenting of the left anterior descending artery. On follow up one year out from stent placement, the patient remained asymptomatic with exercise tolerance and pulmonary function returning to baseline.

Discussion

This is the first report of a cystic fibrosis patient diagnosed with symptomatic coronary artery disease (CAD) and acknowledges that adult cystic fibrosis patients have indeed survived into the years were coronary artery disease becomes prevalent. Therefore, the likelihood of coronary disease should be included in the diagnostic consideration of persistent dyspnea associated with chest discomfort by applying the same standards used in grading a differential with an anginal presentation in non-CF patients.

References

  1. Onady, G.M., Ferinet, C.L. An adult cystic fibrosis patient presenting with persistent dyspnea: case report. BMC Pulmonary Medicine. 2006 May; 6(9): 1-4. Retrieved March 23, 2014. Available at http://www.biomedcentral.com/1471-2466/6/9