Words By Chloe Groth
Ehlers-Danlos Syndrome (EDS) may cause cardiac complications far more than what is often perceived. What is it and why is it a condition that isn’t well understood or researched in cases of traumatic injuries? With significant effects to mobility and organ functionality, his condition needs to be further highlighted in the pre-hospital setting.
EDS is a genitive connective tissue disorder which affects collagen formation and function within the body. This can then affect multiple organ systems. Several complications may arise with this syndrome such as joint dislocations, chronic pain, fatigue, arterial ruptures, and organ ruptures (1). In addition to this, research has also shown links between acute cardiogenic pulmonary oedema and pneumothorax or hemothorax are other potential and pre-existing complications associated with EDS (2,3). However, this could be seen as a limitation due to the lack of evidence, presentation, and epidemiology of such a complication. This is understandable as there are 13 subtypes of EDS. Some of these subtypes include Classical EDS (cEDS), Classical-like EDS (clEDS), Cardiac-valvular (cvEDS), and vascular EDS (vEDS).
Case Study
An 18-year-old male patient that presented as pale diaphoretic, and SOB following blunt force trauma to the thoracic area. There is myriad of other differential diagnosis for this patient, such as pneumothorax, tension pneumothorax, fractured ribs, and pulmonary contusions. The list can go on without further diagnostics. For me, this patient was suffering from an acute cardiogenic pulmonary oedema. The patients’ vitals consisted of HR 145-150bpm, BP - 74/40weak bilateral radial pulses, SPO2 84%, wheezes/ crackles in the left lower lobes, abnormal heart sound 3, pale, cool temp of 35, diaphoretic, chest pain described as heaviness and tight, SOB, diaphragmatic breathing, ECG - tachycardic and T-wave inversions. The t-wave inversions were noted as previous hx changes in the patient’s ECG’s. In alignment with the presenting trauma, abrasions to the patient’s chest, arm and back were noted. There were no obvious deformities to the thoracic, abdomen, shoulders, neck or arms. After a conversation with the patients mother who was on scene she stated that there was a note of previous wandering atrial pacemaker events that were not captured adequately to be diagnosed as yet with specific ECG changes of T-wave depression.
The initial management of this patient consisted of oxygen therapy at 10L/min (SPO2 increasing to 94%), IV access for morphine and fluids administration which was consulted due to the patients deteriorating BP and primary diagnosis of pulmonary oedema. The potential to exacerbate the patients BP and possible pulmonary oedema was taken into consideration. Therefore, treatment was done slowly and with continuous reassessments, especially chest auscultations.
The patient was then transferred over to an Ambulance Service for transportation to the closet trauma hospital for further assessment and management.
The understanding of the presenting case suggests that blunt force trauma had resulted in thoracic injuries such as pulmonary contusion, diaphragmatic injuries, myocardial injury and thoracic aortic disruption. With many risks factors that may affect the morbidity and mortality in blunt chest trauma such as age and pre-existing conditions such as connective tissue disorders (4). It is unclear whether the trauma resulted in heart failure causing contusion of the myocardial muscles, rupture of a cardiac chamber of disruption of heart valves or vessels (5) which could be seen to have increased the progressiveness of the patients symptoms due to the frailty of the patient’s EDS affected myocardium. A follow up and further research would be necessary to clarify this. It should also be noted that poor vascular tone, inability to maintain pressure, and limited vascular tone resulted in further deterioration of the patient. Without adequate knowledge of the complications that can occur with EDS, it would be unclear if the patient would have deteriorated more quickly, nor the potential changes to the patient’s outcome. Finally, there may be links to possible acute and chronic aortic regurgitation with connective tissue disorders. This may cause symptoms of pulmonary oedema, arrhythmias, and heart failure, all of which could be part of the patient’s diagnosis for the presenting complaint (6).
References:
1. Miklovic, T. & Sieg, V. (2021). Ehlers Danlos Syndrome. Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK549814/
2. Sovari, A., Kocheril, A & Baas, A. (2020). Cardiogenic Pulmonary Edema. Retrieved from: https://emedicine.medscape.com/article/157452-overview#a6
3. Chohan, K., Mittal, N., McGillis, L., Lopez-Hernandez, L., Camacho, E., Rachinsky, M., Mina, D., Reid, D., Ryan, C., Champagne, K., Orchanian-Cheff, A., Clarke, H., Rozenberg, D. (2021). A review of respiratory manifestations and their management in Ehlers-Danlis syndromes and hypermobility spectrum disorders. Retrieved from: https://journals.sagepub.com/doi/full/10.1177/14799731211025313
4. Traumatol, C. (2020). Blunt trauma related chest wall and pulmonary injuries: An overview. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7296362/
5. Weiser, T. (2022). Aortic Disruption (Traumatic). Retrieved from: https://www.msdmanuals.com/en-au/professional/injuries-poisoning/thoracic-trauma/aortic-disruption-traumatic
6. Armstrong, G. (2021). Aortic Regurgitation (Aortic Insufficiency). Retrieved from: https://www.msdmanuals.com/en-au/professional/cardiovascular-disorders/valvular-disorders/aortic-regurgitation