HCA Florida Aventura Hospital
Pulmonary Critical Care
pulmonary hypertension, aortic coarctation
Cardiovascular Diseases | Critical Care | Medicine and Health Sciences
Introduction: Aortic coarctation has an incidence of 0.04% and is typically amendable either through a surgical or interventional approach. Unfortunately, patients that have undergone repair of the coarctation are at risk of systemic hypertension which suggests a more diffuse vasculopathy. The arteriopathy can also affect the pulmonary vasculature leading to pulmonary hypertension (PH). The development of PH is a poor prognostic factor. Patients with a history of coarctation repairs have more significant afterload measurements which stiffen their left ventricle and decrease compliance and diastolic function. Adults with Coarctation of the aorta (CoA) are at increased risk of premature cardiovascular death or heart failure secondary to persistent subendocardial fibrosis. We present a case of pulmonary hypertension in a patient with CoA status post surgical aortic valve replacement (SAVR).
Case Presentation: Our patient is a 35 years old female with a history of PFO, ASD, HTN, subclavian stenosis, and CoA who presented with 6 months of worsening dyspnea. The patient underwent SAVR with bioprosthesis twice. Workup included a chest x-ray denoting cardiomegaly and pleural effusions. A new 6/6 systolic murmur was discovered on exam. CTA chest displayed a 4.7 cm ascending aorta with multiple saccular aneurysms. 2D echocardiogram was significant for moderate to severe AS, no TR, and normal PAP. Her workup was positive for hemolytic anemia suggestive of blood vessel vs cardiac valve related hemolytic anemia requiring transfusion. An elective cardiac catheterization was performed due to persistent dyspnea and was positive for severe PH with a PCWP 44 mmHg. The patient was referred to CT surgery for further evaluation for a redo SAVR.
Conclusion: Our case emphasizes the complexities involved in managing CoA and mitigating the development of pulmonary hypertension in spite of SAVR. The underlying pathophysiology linking CoA and PH and its risk of increased mortality warrants further investigation.
Devine, Adam and Garcia, Jacklyn, "Persistent Challenges: Pulmonary Hypertension in Aortic Coarctation Despite Surgical Valve Replacement" (2023). East Florida Research Day 2023. 34.