SYNCOPE IN A 20 YEAR OLD FEMALE: AN UNUSUAL ETIOLOGYMALLORY BROWNING

OMS IV

WILLIAM CAREY UNIVERSITY COLLEGE OF OSTEOPATHIC MEDICINE

DISCLOSURES

• None

INTRODUCTION

SYNCOPE

• Common chief compliant in ED and occurs in all age groups

• Syncope has any number of causes including those arising from a physiologic and anatomical component

• In those with prior noted anatomical deformities, these deformities should be considered and evaluated as a potential cause of their syncopal episodes

CASE PRESENTATION

PATIENT PRESENTATION

• 20 year old female presented to the ED with chief complaint of syncope

• Patient was teaching dance class and had a syncopal episode while bending over

• Intermittent, having occurred numerous times in the past +/- associated chest pain

• Numerous past work ups for syncope • Aggravated by being stressed, hot, or exerting herself

PATIENT PRESENTATION

• Presented to numerous outside clinics with the same complaint

• Reported syncope or near syncope every day prior to current ED visit

• Work ups were performed at these facilities leading to a diagnosis of exclusion of panic disorder/anxiety

• However, medical history indicated the patient had recently been limiting her activity level secondary to near syncope and dyspnea

PAST MEDICAL HISORY

• Pectus excavatum diagnosed at age 13 and cleared by a pediatric cardiologist

• Panic Disorder/Anxiety patient’s syncopal episodes were attributed to panic attacks

PHYSICAL EXAM

• Vital Signs: BP 124/59, HR 86 bpm, RR 20, temperature 98.5 degrees F, oxygen saturation 98.5% on room air

• Patient was in no distress, appears well, and well hydrated

• Chest wall upon initial evaluation showed no obvious deformity

• However, during examination while standing at the foot of the bed it was noted the patient’s left rib cage is significantly higher than the right rib cage

• Of note the patient’s body habitus did not allow for immediate identification of how significant her pectus deformity was

DIFFERENTIAL DIAGNOSIS

• Intrauterine pregnancy

• Ectopic pregnancy

• Rhythm disturbance

• Orthostatic hypotension

• Electrolyte abnormality

• Anemia

• Pulmonary embolism

• Other anatomic intrathoracic cause

WORK UP—LABS

• EKG-normal sinus sinus rhythm

• CBC-unremarkable

• CMP-unremarkable

• Pregnancy test-negative

• D-dimer <150 ng/mL

WORK UP—IMAGING

• CXR shows no acute cardiopulmonary disease, discusses notable pectus excavatum defect in radiology report

• Last CXR performed in 2009, current x-ray does not show any significant changes

• CTA ordered after discussing with patient and mother• Patient noted to have had prior CT performed in 2012

WORK UP--IMAGING

WORK UP—IMAGING

• Severe pectus excavatum deformity of the anterior chest wall was noted

• The lower sternum and xiphoid process produced mass effect upon the IVC and right atrium

• This mass effect worsened as compared to prior CT chest from 2012.

PATIENT COURSE

• Admitted and evaluated by cardiologist and cardiothoracic surgeon

• Cardiology performed echo showing EF of 55-60 % without IVC impingement

• CV Surgery evaluated using the Haller Index w/ a calculated score of 4

• Patient referred to a tertiary care center for surgical correction of defect with a Ravitch procedure• No wound complications

DISCUSSION

PECTUS EXCAVATUM

• Isolated cardiac disease, symptoms, or mechanical damage solely attributable to pectus excavatum defects are relatively rare

• When they do occur they are generally secondary to an inherited genetic disease such as Marfan’s or Noonan’s syndrome(s)

• Interestingly 30% of pectus defects can worsen during an adolescent’s growth spurt • No definitive method to predict their progression or predict how severe it may be

HALLER INDEX OR PECTUS SEVERITY INDEX (PSI)

HALLER INDEX

• Standard Haller index is <2.5

• Pectus deformity of 3.25 are those referred for corrective surgery

• Those with a Haller index of 3.25 or greater and cardiorespiratory compromise will require imaging to assess for internal organ compression and the need for additional studies• EKG-–may show right heart strain in 68 % of female patients

• ECHO

• Right axis deviation, if seen, may indicate that the deformity is severe enough to rotate and compress the heart to that degree

SURGICAL CORRECTION

• Indicated in those with a Haller index of >3.5

• Modified Ratvich

• Nuss Procedure

• Historically, prior to the modified Ratvich and Nuss procedures, pectus defects with cardiorespiratory compromise required radical resections of the rib cage and other cardiothoracic structures

SURGICAL CORRECTION

• Nuss Procedure• Decreases sternal stress• Incise thoracic/rib cage cartilage and use lateral stabilizers to reinforce the rib cage• Longer and more complicated defects are treated by using multiple Nuss bars,

removed after two years, generally at the discretion of the surgeon who placed them

• Modified Ratvich Procedure• Variation on Brown’s method • Resection of the cartilage w/ a sternal osteotomy under the angle of the pectus

defect • Then the sterum is temporarily fixed internally to provide support

OTHER METHODS OF CORRECTING PECTUS EXCAVATUM

• Sternal suction

• Sternal magnets

• Prosthetic inserts

• Physical therapy

CONCLUSION

KEY POINTS

• Rule out any acutely life threatening conditions with a presentation of syncope, considering the patient’s age and associated conditions

• DO NOT assume prior assessments are accurate

• DO NOT let prior assessments bias your clinical decision making

• Perform a thorough clinical exam

• When dealing with pectus excavatum know the proper testing and how to interpret the Haller score

• Keep in mind any underlying genetic conditions that can contribute to a pectus excavatum defect

REFERENCES

• Mayer, Oscar H. “Pectus Excavatum: Etiology and Evaluation.” UpToDate, 2017, www.uptodate.com/contents/pectus-excavatum-etiology-and-evaluation?search=pectus%20excavatum%20etiology%20and%20evaluation&source=search_result&selectedTitle=1~46&usage_type=default&display_rank=1.

• Mayer, Oscar. “Pectus Excavatum: Treatment .” UpToDate, 2019, www.uptodate.com/contents/pectus-excavatum-treatment?search=pectus%20excavatum%20treatment&source=search_result&selectedTitle=1~46&usage_type=default&display_rank=1#H6.