Arteriopathy is a cardiovascular disease that is linked to several genetic conditions — including Williams syndrome, Ehlers-Danlos syndrome (EDS), and Marfan syndrome — and the vascular disease midaortic syndrome. While symptoms vary, the syndromes have a common denominator: They are connective tissue disorders.
Arteriopathy is when the weakened connective tissues prevent the heart’s arteries — including the main artery, the aorta — from effectively pumping oxygen-rich blood throughout the body and depriving other vital organs from functioning properly.
The Arteriopathy Program at Boston Children’s brings together cardiac surgeons and cardiologists from the hospital’s Benderson Family Heart Center along with general surgeons, pulmonologists, and other specialists to collaborate on the care of children with arteriopathy. We particularly work close with the Center for Cardiovascular Genetics so we can approach a patient’s arteriopathy treatment as part of a comprehensive care plan that also considers the treatment of other conditions associated with the genetic syndromes.
Our approach to arteriopathy treatment
Most children with arteriopathy require cardiology care, and many also need surgery. No matter how we approach a child’s care for arteriopathy, it’s from a position of experience that allows us to create treatments and update proven conventional ones — all to improve their blood flow and quality of life.
Because the genetic syndromes affect the aorta and other arteries in different ways, our team of specialists will create the most appropriate individualized care plan to best treat your child’s arteriopathy. Here are some condition-specific approaches we might take:
Williams syndrome
- collaborations between cardiac surgeons and cardiologists to use groundbreaking techniques that have been proven to effectively rehabilitate the aorta and coronary and pulmonary arteries
- catheterization of blood vessels by an interventional cardiologist: either through ballooning (a balloon stretches the constricted vessel) or stenting (a special tube that holds open a narrowed artery)
- a hybrid approach in which an interventional cardiologist and cardiac surgeon place stents in difficult-to-reach blood vessels
Marfan and Ehlers-Danlos syndromes
- cardiology care to prescribe medication, including beta blockers, to lower the child’s blood pressure and reduce stress on the aorta, and angiotensin II blockers such as Losartan
- cardiac surgery to replace the affected part or parts of the aorta and/or to repair the heart valves if they are not working properly
- surgeons from our Congenital Heart Valve Program will attempt to preserve the native valve of a child with Marfan’s syndrome by performing a valve-sparing aortic root operation
Midaortic syndrome
- cardiology care to manage medications to control blood pressure and improve heart function
- catheterization for minimally invasive endovascular treatments that offer faster recovery times, such as angioplasty or stenting
- cardiac or vascular bypass graft surgery that develops paths for blood flow by creating detours around narrowed arteries
