1. DIAGNOSTIC DILEMMA : NEARLY MISSED !!!!!
By :
Prof. Dr. : Fawzy Megahed

2. A 37-year-old woman who had been previously healthy, was 1 week postpartum when she presented to the emergency department with chest pain, shortness of breath, and lower extremity swelling.

3. Her vital signs were normal, and the physical examination showed mild pitting edema.

4. What are your differential diagnosis ?

5. Which one of the following is the least likely diagnosis ?
1 – Peripartum Cardiomyopathy 2 – Pulmonary Embolism 3 – Acute Myocardial Infarction 4 – Pre-eclampsia 5- Postpartum Thyroiditis 6 – Pericardial Effusion

6. Computed tomography angiography (CTA) for pulmonary embolism was negative. A transthoracic echocardiogram (TTE) showed an ejection fraction of 65% without regional wall motion abnormalities.

7. Subsequently, she was discharged home with a diuretic.

8. One week later, she returned with sudden onset of sharp, “knife-like” flank pain radiating up her back. This was accompanied by chest pain and a severe headache. A review of systems was otherwise negative.

9. At her second visit to the emergency department, the patient’s vital signs were as follows: blood pressure, 186/108 mmHg; heart rate, 68 beats per minute; temperature, (36.8_C), and respiratory rate, 14 breaths per minute. Her oxygen saturation was 98% on room air.

10. Cardiopulmonary and abdominal examinations were normal
Cardiopulmonary and abdominal examinations were normal. Peripheral pulses were palpable and symmetric.

11. Laboratory data, urinalysis, and electrocardiogram were unremarkable
Laboratory data, urinalysis, and electrocardiogram were unremarkable. CT without contrast of the abdomen and pelvis failed to reveal acute pathology.

12. Which one of the following is the most likely diagnosis ?
1 – Marantic Endocarditis With Embolisation 2 – Pre-eclampsia 3 – Systemic Lupus Erythematosis 4 – Pheochromocytoma 5- Fibromuscular Dysplasia Of The Renal Artery 6 – Other diagnosis

13. Preeclampsia was suspected at this point, and the patient received magnesium sulfate in the emergency department and an antihypertensive regimen. She was discharged home with a plan to obtain an outpatient TTE.

14. One week later, she underwent the imaging, which was concerning for aortic pathology.

15. ?

16. One week later, she underwent the imaging, which was concerning for aortic dissection.

17. CTA confirmed a type B aortic dissection without malperfusion
CTA confirmed a type B aortic dissection without malperfusion. The dissection extended from the origin of the left subclavian artery to below the right common femoral artery .

18. Figure Computed tomography angiography was performed
Figure Computed tomography angiography was performed. This coronal view of the aorta shows acute type B aortic dissection, which begins just distal to the origin of the left subclavian artery and extends to the distal aspect of the right common femoral artery.

19. Diagnosis
Acute aortic dissection is an extremely rare and often fatal disease, with an estimated annual incidence of 30 cases per million people. The condition is even rarer among women in the postpartum period, and medical and surgical management in these patients has not been well studied.

20. Relative hemodynamic stressors, including increased heart rate, stroke volume, and cardiac output, are normal physiologic responses during pregnancy. Compression of the aorta during the third trimester can spur structural changes and alter hemodynamics.

21. Concurrent hormonal effects may predispose the patient to changes in the microstructure of the aorta, particularly in the intima and medial layers. Animal studies suggest that oxytocin may be associated with pregnancy-induced aortic dissection

22. A detailed history and physical examination can uncover additional underlying risk factors, including a history of prior cardiac surgery, connective tissue disease or vasculitis, and disorders caused by inherited or acquired gene mutations, such as Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, familial aortic aneurysm, Turner’s syndrome, and bicuspid aortic valve.

23. Classification aortic dissection

24. Aortic dissection transesophageal echocardiogram

25. Dissection of the descending aorta on TEE

26. Abdominal aortic dissection

27. Thoracic aortic dissection has been associated with 8 genetic mutations, which occur either in genes involved in transforming growth factor beta (TGFb) signaling or genes encoding smooth muscle-restricted contractile proteins.

28. As in our patient’s case, the most commonly reported symptom in aortic dissection associated with pregnancy is back pain. Dyspnea, syncope, and signs of respiratory distress or circulatory compromise also have been described.

29. Our patient presented with significant hypertension, a sign observed in 70% of patients with type B aortic dissection. Selection of imaging modalities depends on patient stability, renal function, and accessibility of technology.

30. CT or magnetic resonance imaging is preferred, as the modalities provide > 98% diagnostic accuracy for aortic dissection.

31. Management
The patient was admitted to the surgical intensive care unit for blood pressure control using intravenous antihypertensive agents (systolic blood pressure goal, < 120 mmHg). Once she transitioned to oral antihypertensive drugs, a repeat CTA was ordered, and this showed stable type B aortic dissection.

32. Subsequently, she was discharged home on amlodipine, lisinopril, and metoprolol succinate. Outpatient genetic testing and connective tissue disorder screening proved negative. Her surveillance imaging has remained stable.

33. Management of the peripartum patient with aortic dissection requires a multidisciplinary approach by internists, cardiologists, obstetricians, and cardiothoracic surgeons.

34. To limit aortic wall stress in patients who do not require surgery, treatment begins with an intravenous beta-blocker (systolic pressure goal, < 120 mmHg); if the patient is unable to tolerate beta-blocker therapy to achieve heart rate control, treatment can start with a calcium channel blocker.

35. Then, if rate control is achieved but the blood pressure goal has not been reached, a vasodilator—nitroprusside, for example—is added. While other agents, such as an ACE inhibitor or i.v. CCBs can be added if necessary, hydralazine should not be used.

36. To avoid reflex tachycardia, a vasodilator should not be prescribed until after rate control is obtained. Also, analgesics are administered early. Further, it is imperative to note that achieving blood pressure control is more selective during pregnancy, as certain medications, such as atenolol and ACE inhibitors, are contraindicated.

37. Further, it is imperative to note that achieving blood pressure control is more selective during pregnancy, as certain medications, such as atenolol and ACE inhibitors, are contraindicated.

38. Surveillance imaging to monitor aorta size is recommended at 3-, 6-, and 12-month intervals during the first year. If the aortic size is stable, annual surveillance is sufficient thereafter.

39. Unlike our patient, one third of patients presenting with type B aortic dissection develop complications requiring intervention.

40. Endovascular stent-grafting of the descending thoracic aorta has become the gold standard therapy for patients who develop refractory pain, uncontrolled hypertension despite medical therapy, malperfusion syndromes, or contained or impending rupture.

41. Pregnant patients with a baseline aortic root size greater than 4 cm should undergo surveillance TTE every 4-8 weeks for close monitoring of aneurysmal progression.

42. While the need for surgical intervention must be weighed for each patient on an individual basis, surgery should be considered once the aortic diameter is larger than 4.5 cm, depending on various risk factors or if rapid progression is evident (size increases by more than 3-5 mm in a year).

43. Timing of the surgery largely depends on the patient’s stability and the viability of the fetus. Preconceptual counseling and informed decision-making cannot be overemphasized for patients with underlying risk factors.

44. Pregnancy should be avoided in women with a high risk for dissection or rupture. If these patients wish to become pregnant, elective surgical intervention should be considered, depending on the aortic root size and overall risk factors.

45. Elective surgery should be contemplated when the aortic root is larger than 4 cm in patients with Marfan syndrome or if the maximal cross-sectional area of the ascending aorta or root in square centimeters, divided by the patient’s height in meters, exceeds a ratio of 10 in patients with short stature.

46. Patients with mutations in TGFbR1 or TGFbR2 or Loeys-Dietz syndrome can undergo elective surgical repair if TTE shows that the internal aortic diameter exceeds 4.2 cm.

47. Prophylactic repair is also reasonable when the aortic diameter measures more than 4.5 cm in patients who have a history of previous aortic valve surgery, a connective tissue disorder, a family history of aortic dissection, or growth of the aortic diameter exceeding 0.5 cm per year.

48. In patients without risk factors, aortic repair is recommended when the aortic diameter exceeds 5.5 cm. Women with any of the above risk factors for an aortic diameter greater than 4 or 4.5 cm should undergo a thorough work-up to determine whether they are candidates for aortic repair prior to pregnancy.