Total pulmonary arterial reconstruction in a patient with arterial tortuosity syndrome affecting the pulmonary artery: a case report and review of the literature.

BACKGROUND: Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events. CASE PRESENTATION: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg. CONCLUSION: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

Hybrid approach to management for a patient with culture negative infective endocarditis with profunda femoris mycotic pseudoaneurysm: A case report and review of the literature.

Mycotic pseudoaneurysms can be a serious and life threatening complication of left sided infective endocarditis. They most commonly affect the major axial vessels. Profunda femoris artery (PFA) aneurysms are rare and present in only 0.5% of all peripheral aneurysms, regardless of the underlying etiology. We present a case of a patient who underwent mitral valve repair for severe mitral regurgitation secondary to culture negative IE which was complicated by multiple mycotic pseudoaneurysm. The PFA pseudoaneurysm which was affected and was complicated with a large hematoma compressing the femoral nerve. This was managed by a staged hybrid approach. Endovascular stenting was performed first to seal the pseudoaneurysm and facilitate open surgical repair using a reversed interposition saphenous vein graft. To the best of our knowledge, this is the first reported case of a PFA mycotic aneurysm (MA) being managed by a hybrid approach using endo-vascular and open surgical repair. MAs and pseudoaneurysms are complex and life threatening conditions requiring meticulous planning for optimal management. Endovascular stenting can be considered as an alternative to surgical management in certain cases or as a bridge to definitive open surgical repair depending on anatomical location and associated complications.

Aortic root abscess and Brucella endocarditis in a patient with mechanical aortic valve prosthesis: a case report.

Rare but potentially fatal, brucellosis prosthetic valve endocarditis is a complication of brucellosis caused by Brucella species. The symptoms of brucellosis can be nonspecific, making the diagnosis challenging. Osteoarticular involvement is the most common complication of brucellosis. Mortality from brucellosis is low except for endocarditis and involvement of the central nervous system. The diagnosis is based on laboratory tests and clinical manifestations. Serological tests are preferred, as culture methods can be unreliable. A 59-year-old woman presented with gastrointestinal bleeding, fever, anorexia and malaise. She had a history of aortic valve replacement with a mechanical prosthesis for severe bicuspid aortic stenosis. Investigations revealed a multiloculated aortic root abscess encircling the prosthetic valve. She was diagnosed with brucella endocarditis, treated with antibiotics and underwent cardiac surgery. Her symptoms improved following the surgery. Brucellosis prosthetic valve endocarditis is a rare presentation of this disease.