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Thoracic Aortic Dissection Combined with Dilated Cardiomyopathy in a Patient with Autosomal Dominant Polycystic Kidney Disease

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Thoracic Aortic Dissection in a Patient with Autosomal Dominant Polycystic Kidney Disease: A Case Report

HK Lamba MD, S Plato MD, Y Elgudin PhD

Department of Surgery Ÿ University Hospitals Case Medical Center

 

INTRODUCTION

-Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited disorders in humans and is responsible for 6–10% of cases of end stage renal disease.
-The leading cause of morbidity and mortality in ADPKD is cardiovascular disease.
-Cardiovascular abnormalities in ADPKD including most commonly hypertension and less commonly heart valve lesions, cardiomyopathy, cerebral aneurysms, aortic root dilation, and abdominal aortic aneurysm.
-Thoracic aortic dissection has been rarely reported and ascending thoracic aortic dissection even more rarely.
 

HISTORY AND PHYSICAL

-34 year old white male with end stage renal disease (ESRD) secondary to autosomal dominant polycystic kidney disease (ADPKD).
-Previously admitted at an outside hospital (OSH) for dyspnea on exertion (DOE) and greyish sputum
-Transthoracic echocardiography at the OSH demonstrated possible vegetation and ejection fraction (EF) of 20%
- Treated for culture negative endocarditis  and new onset heart
-Presented again with 1 month history of worsening DOE, lightdeadedness, and chest pressure.
-Transesophageal Echoardiograph showed linear opacity in the proximal aorta suggestive of focal dissection and EF of 25%
 

PROCEDURE

-TEE and CTA findings were consistent with type A dissection and patient was taken to the operating room for ascending aorta replacement with tube graft.
-Placed on cardiopulmonary bypass (CPB) using right axillary cannulation.
-Median sternotomy performed
-Transection of ascending aorta revealed dissection of the non-coronary sinus with chronic flap in the lumen.
-Dissection extended into the right coronary sinus without involving the right coronary artery.
-Leaflets of the aortic valve preserved with no evidence of endocarditis.
-Valve sparing aortic root replacement (David procedure) with a 28 mm Valsalva graft and 26 mm Hemashield graft performed.
-Dissections of right and left coronary buttons glued with BioGlue before re-insertion onto graft.
-Successfully weaned off CPB in normal sinus rhythm.
 

POSTOPERATIVE COURSE

-Thrombocytopenia and coagulopathy on Postoperative Day (POD) 2 requiring transfusio
-Discharged to home on POD 12
-Genetic studies showed no evidence of Marfan’s disorder.
-CTA on POD 76 demonstrated normal course and caliber of aorta.
 

DISCUSSION

-We report a rare constellation of symptoms associated with ADPKD; polycystic kidney disease, dialted cardiomyopathy, and ascending thoracic aortic dissection.
-ADPKD presenting with cardiovascular abnormalities is often associated with concomitant Marfan’s Disorder, a genetic connective tissue disorder.
-Interestingly, our patient did not meet clinical or genetic diagnosis of Marfan’s.
-Mutations of the PKD1 and PKD2 genes found in 85% and 15% of cases of ADPKD.
- PKD1 mutations result in Polycystin-1 deficiency, which is present in normal endothelium, possibly contributing to loss of vascular structural integrity.
-PKD2 mutations result in Polycystin-2 deficiency, which normally modulates intracellular calcium cycling, possibly contributing to the development of heart failure.  
 

REFERENCES

 

1. Gabow PA. Autosomal dominant polycystic kidney disease: More than a renal disease [Review]. Aiiz J Kidney Dis 1990;16:403–413.

2.  Smith MK, Metcalfe M, DeMaria, Hendren WG, Walter PF, Jones EL. Hypereosinophilic Syndrome Resulting in Aortic and Mitral Stenosis: A Case Requiring Double Valve Replacement. American Heart Journal.  Feb 1989 117(2):475-479.

3.  Kang YR Multiple Cardiovascular Manifestations in a Patient with Autosomal Dominant Polycystic Kidney Disease J Cardiovasc Ultrasound 2014 Sep: 22(3):144-147

4. Paavola J Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy J Mol Cell   Cardiology 2013 May;58:199-208.

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