In simple words: This code indicates that a person has Marfan syndrome, a genetic condition that affects connective tissue, and they are experiencing heart-related problems other than a widened aorta. These problems can involve the heart valves or other blood vessels.

Marfan syndrome is a genetic disorder that affects the body's connective tissue. This code specifies that the individual has Marfan syndrome along with cardiovascular manifestations other than aortic dilation. Marfan syndrome can weaken the connective tissue around the heart and blood vessels. It most commonly affects the aorta, the large blood vessel that carries blood from the heart to the rest of the body.The aorta can widen or bulge, a condition called an aneurysm. It can also tear (aortic dissection), which can be life-threatening. Marfan syndrome can also affect the heart valves, causing them to leak or not close properly. This can lead to shortness of breath, fatigue, and other symptoms.

Example 1: A 25-year-old patient with a confirmed diagnosis of Marfan syndrome presents with mitral valve prolapse and tricuspid regurgitation. Both are documented in the patient’s medical record., A 40-year-old individual with a family history of Marfan syndrome exhibits symptoms like shortness of breath, chest pain, and heart palpitations. An echocardiogram reveals pulmonary artery dilatation. Genetic testing later confirms Marfan syndrome., A child with Marfan syndrome undergoes regular checkups, and during one such visit, the physician detects a heart murmur not related to aortic dilation. Further investigation reveals a ventricular septal defect.

Documentation should include a clear statement of the diagnosis of Marfan syndrome. In addition to that, detailed clinical findings related to the specific cardiovascular manifestations, results of diagnostic tests (e.g., echocardiogram, ECG), and any related procedures performed should also be clearly documented.