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5 Case 1-2013: A 72-year-old female with Tetralogy of Fallot and increasing dyspnea

A 72-year-old woman with an uncorrected tetralogy of Fallot presenting with possible pulmonary endocarditis: a case report. Journal Of Medical Case Reports, 7(1). 2013. doi: 10.1186/1752-1947-7-150

Sousa, P., Santos, W., Marques, N., Cordeiro, P., Ferrinha, R., & Pereira, S. et al.

Case Summary 1

A 72-year-old Caucasian female with complaints of dyspnea was admitted to the hospital with symptoms of fever and heart failure. The patient has a history of controlled hypertension and healthy pregnancy. Prior to this hospital admission, she had started treatment of dental caries and tooth extraction with the use of prophylactic antibiotics. Physicians had conducted cardiac magnetic resonance imaging, a transthoracic echocardiogram, and laboratory investigations.

 


Clinical History 1

Learning Objectives

  • Investigating the clinical history of the patient and selecting appropriate examinations for the diagnosis of tetralogy of Fallot (TOF).
  • Understanding the criteria for possible diagnosis and identifying those presented in the patient.
  • Familiarizing and defining new medical terminology associated with the disorder.
  • Extrapolating key lifestyle factors that have contributed to other diseases.
  • Age:  72 years old
  • Sex:  Female
  • Ethnicity:  Caucasian

Medical History 1

Symptoms 1

  • Recurrent attack of persistent vespertine low-grade fever for 5 months.
  • Weight loss and asthenia for same duration.
  • Gradually dyspnea (class III NYHA – Comfortable at rest, limitations in physical activities and <ordinary activities cause palpitation, dyspnea and fatigue.)

 

Examinations (Clinical Assays/Tests/Imaging) 1

Physical Examination 1

  • Slight peripheral cyanosis (blue extremities due to oxygen-poor blood)
  • Finger clubbing
  • Oxygen saturation of 91%
  • Pulse rate 95 beats per minute
  • Blood pressure of 149/70 mmHg

Cardiac Examinations 1

  • Single second heart sound.
  • A systolic thrill and loud systolic ejection murmur (grade IV – associated with thrill and easily auditable) at the base of her heart.

Blood Investigations 1

  • Blood results showed leukocytosis (WBCs: 17, 700), elevation of C-reactive (inflammatory marker) protein with high sedimentation rate (indication of high inflammatory disease) and rheumatoid factor (RF – immune proteins that attack healthy tissues in body) and mild anemia (hemoglobin 11.7 g/L and hematocrit 0.35 L/L).
  • No vascular or immunological phenomena (except RF) were detected.
  • All blood cultures were negative inclusively for atypical organisms.
  • Serology tests were negative.
  • Showed normal sinus rhythm (normal and healthy impulses of the heart) with first degree atrioventricular block (slow conduction of atrioventricular node) and incomplete right bundle branch block (delay in pulse electrical pathway to conduct heart beat).

Cardiac Magnetic Resonance Imaging (MRI) 1

  • Revealed a large subaortic VSD, diameter of 26 mm and bidirectional flow (QP:QS = 0.9)
  • An overriding of the aorta over the septum ≤50% (abnormal positions of aorta above VSD)
  • A marked hypertrophy (thickness) of the right ventricle (RV) with subpulmonary stenosis (blockage due to dense muscle fibers) (max. gradient of 31 mmHg)
  • Moderate pulmonary regurgitation (leaky pulmonary valve).
  • A dilation of the pulmonary artery and its branches.
  • A nondilated left ventricle with preserved systolic function.
  • No patent ductus arteriosus (a condition where ductus arteriosus fails to close at birth) was present.
Cardiac magnetic resonance images
Figure 1: Cardiac magnetic resonance imaging revealing condition of tetralogy of Fallot. “A: Ventricular septal defect and overriding aorta over the septum. B: Right ventricular hypertrophy and subpulmonary stenosis.” 1

Transthoracic Echocardiography (TTE) 1

  • Showed the components of TOF and an echo dense, irregular and mobile mass, 10 mm long and 3mm wide, adherent and downstream to the pulmonary valve suggestive of vegetation (mass due to bacteria growth on a heart valve) without associated regurgitation (leaking of heart valve).
  • Thus, she was hospitalized for suspected infective endocarditis.
  • After 4 weeks of antibiotic therapy, no images suggestive of vegetation were detected on TTE (Figure 4).
Echocardiography images
Figure 2: Tetralogy of Fallot diagnosis supported using transthoracic echocardiogram. “A: Five chamber view with the presence of the ventricular septal defect (VSD) and the overriding aorta over the septum. B: Parasternal long axis view revealing the VSD, the overriding of the aorta over the septum and also the right ventricle (RV) hypertrophy. AO, aorta; IVS, interventricular septum; LA, left atrium; LV, left ventricle.” 1

 

transthoracic echocardiogram
Figure 3: Suggestive vegetation in pulmonary valve due to erratic structure seen through transthoracic echocardiogram. “AoV, aortic valve; PA, pulmonary artery; PV, pulmonary valve.”1

 

transthoracic echocardiogram images
Figure 4: Pulmonary valve at discharge using transthoracic echocardiogram, 4 weeks after empiric antibiotherapy.1

Transesophageal Echocardiography (TEE) 1

  • Was not able to confirm or exclude the presence of pulmonary vegetation given the complex heart anatomy.

Computed Tomography Images 1

  • Revealed no signs of cerebral, thoracic, or abdominal embolization.

 

Question & Answers Leading to Diagnosis: 

Question 1: The patient’s clinical history includes a tooth extraction, recurrent fever and dyspnea, together what could be a possible diagnosis for this patient?

Question 2: Between the physical and imaging examinations, what results would support this patient’s tetralogy of Fallot disease, does she meet all the criteria for this diagnosis?

Question 3: In the TTE, vegetations were apparent in the pulmonary valve, how would this support the previously suggested diagnosis?

** For answers please check the next chapter.

 

Medical terminology/Abbreviations: 

  • Antibiotic prophylaxis – antibiotic usage before surgery or procedure in order to prevent bacterial infection 6
  • Class II NYHA dyspnea – Mild symptoms of shortness of breath 1
  • Hypertension – A condition where blood vessels persistently have raised pressure 5
  • Hypoxia – A condition where the body or an area of the body is deprived of adequate oxygen in the tissues 8
  • Infective endocarditis – Inflammation of the heart that is caused by a fungal or bacterial infection of the heart valves or the inner lining of the heart 4
  • Pulmonary stenosis/outflow – Associated with structurally abnormal or immunocompromised states of the heart 3
  • Subpulmonary stenosis – A condition when there is blockage below the pulmonary valve due to too much muscle (muscular bundles) 9
  • Systolic ejection murmur – It is turbulent blood flow by the obstruction across semilunar valves, arteries, and outflow tracts 7
  • TOF – Tetralogy of Fallot, a form of cyanotic congenital heart disease 1
  • Transesophageal echocardiography – specific type of echocardiogram to look more closely at the heart to examine potential blood clots 10
  • Ventricular septal defect (VSD) – a hole in the wall (septum) that separates the lower chambers (ventricles) of the heart, this is a birth defect of the heart 2

 

References

  1. Sousa, P., Santos, W., Marques, N., Cordeiro, P., Ferrinha, R., & Pereira, S. et al. (2013). A 72-year-old woman with an uncorrected tetralogy of Fallot presenting with possible pulmonary endocarditis: a case report. Journal Of Medical Case Reports, 7(1). doi: 10.1186/1752-1947-7-150
  2. Congenital Heart Defects – Facts about Ventricular Septal Defect | CDC. (2021). Retrieved 22 March 2021, from https://www.cdc.gov/ncbddd/heartdefects/ventricularseptaldefect.html#:~:text=A%20ventricular%20septal%20defect%20(pronounced,(ventricles)%20of%20the%20heart.
  3. Bamford, P., Soni, R., Bassin, L. et al. Delayed diagnosis of right-sided valve endocarditis causing recurrent pulmonary abscesses: a case report. J Med Case Reports 13, 97 (2019). https://doi.org/10.1186/s13256-019-2034-7
  4. Infective endocarditis. (2021). Retrieved 23 March 2021, from https://www.heartandstroke.ca/heart-disease/conditions/infective-endocarditis#:~:text=Infective%20endocarditis%20is%20an%20inflammation,lead%20to%20life%2Dthreatening%20complications.
  5. Hypertension. (2021). Retrieved 23 March 2021, from https://www.who.int/health-topics/hypertension#tab=tab_1
  6. Prophylactic Antiobiotics: Types, Uses, and Administration. (2021). Retrieved 23 March 2021, from https://www.healthline.com/health/prophylactic-antibiotic-premedication
  7. Heart Murmurs. (2021). Retrieved 23 March 2021, from https://www.utmb.edu/pedi_ed/CoreV2/Cardiology/cardiologyV2/cardiologyV24.html#:~:text=a)%20Systolic%20ejection%20murmurs%20(SEM,shortly%20after%20S1%20(pulse).
  8. Samuel J., ranklin C. (2008) Hypoxemia and Hypoxia. In: Myers J.A., Millikan K.W., Saclarides T.J. (eds) Common Surgical Diseases. Springer, New York, NY. https://doi.org/10.1007/978-0-387-75246-4_97
  9. Pulmonary Stenosis | Children’s Hospital of Philadelphia. (2021). Retrieved 23 March 2021, from https://www.chop.edu/conditions-diseases/pulmonary-stenosis#:~:text=Pulmonary%20stenosis%20can%20be%20mild,this%20is%20called%20subpulmonic%20stenosis.
  10. Transesophageal Echocardiogram (TEE). (2021). Retrieved 23 March 2021, from https://www.heartandstroke.ca/heart-disease/tests/transesophageal-echocardiogram-tee#:~:text=A%20transesophageal%20echocardiogram%20(TEE)%20is,the%20structures%20of%20the%20heart.
  11. Mang-de la Rosa, M. R., Castellanos-Cosano, L., Romero-Perez, M. J., & Cutando, A. (2014). The bacteremia of dental origin and its implications in the appearance of bacterial endocarditis. Medicina oral, patologia oral y cirugia bucal, 19(1), e67–e74. https://doi.org/10.4317/medoral.19562
  12. Seraj, S. M., Gill, E., & Sekhon, S. (2017). Isolated pulmonary valve endocarditis: truth or myth?. Journal of community hospital internal medicine perspectives, 7(5), 329–331. https://doi.org/10.1080/20009666.2017.1374808
  13. C-Reactive Protein Test: Purpose, Procedure, and Results. (2021). Retrieved 26 March 2021, from https://www.healthline.com/health/c-reactive-protein
  14. Blood Culture | Lab Tests Online. (2021). Retrieved 26 March 2021, from https://labtestsonline.org/tests/blood-culture#:~:text=Blood%20culture%20sets%20that%20are,additional%20testing%20may%20be%20required.
  15. Tetralogy of Fallot – NORD (National Organization for Rare Disorders). (2021). Retrieved 12 May 2021, from https://rarediseases.org/rare-diseases/tetralogy-of-fallot/

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This post is adapted for the Health & Medical Case Studies created by the Master of Medical Biotechnology program of the University of Windsor. This work licensed under a Creative Commons Attribution-NonCommercial (CC BY-NC-ND) 4.0 International License.

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Case 1-2013: A 72-year-old female with Tetralogy of Fallot and increasing dyspnea Copyright © 2023 by Sousa, P., Santos, W., Marques, N., Cordeiro, P., Ferrinha, R., & Pereira, S. et al. is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, except where otherwise noted.

Digital Object Identifier (DOI)

https://doi.org/10.1186/1752-1947-7-150

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