Editorial

Coarctation of the aorta, biscuspid aortic valve and abnormal ascending aortic wall

Pathology of sudden death and clinicopathologic correlations are treated in this chapter. Definition, epidemiology, and pathogenetic mechanisms are first addressed. Sudden death is mostly cardiovascular and arrhythmic in origin, with ventricular fibrillation as the precipitating mechanism of cardiac arrest. The majority of cases present with a pathologic substrate at the level of coronary arteries, myocardium, valves, conduction system, and great arteries. However, there are cases without substrate (ion channel diseases) which require molecular investigations in search of transmissible, genetically determined disease. The chapter is mostly devoted to sudden death in the young, with particular reference to the athletes. Anatomy and pathology of the cardiac conduction system is also dealt with, including ECG correlations. AV block and ventricular preexcitation are taken into detailed consideration. Atrial fibrillation, its causes, substrates, potential complications, and treatment are also extensively treated. Guidelines and useful recommendations for postmortem study of sudden death appear at the end of the chapter.

Congenital malformations of the aorta are a heterogenous group of conditions that arise due to either embryological or vessel wall architectural defects. These congenital aortic anomalies may be divided into three main groups. The most common group includes diseases that obstruct blood flow to the distal circulatory system and the classical representative of this category is aortic coarctation. The second category includes those diseases that obstruct either the trachea or the esophagus. Right aortic arch (RAA) is a representative of this category. A third category of congenital malformations of the aorta includes abnormalities of the mechanical composition of the aorta. Classically Marfan syndrome is representative of this category. However, bicuspid valve and its associated aortopathy also qualify for this category and will be the focus of this chapter along with aortic coarctation and RAA. Congenital malformations of the aorta carry a significant morbidity and mortality due to delayed diagnosis, missed diagnosis, or inadequate treatment. Newer developments in endovascular technology, improved understanding of the pathobiology, emerging imaging strategies, and evolving indications to treat may potentially reduce the morbidity and mortality associated with this heterogenous group of conditions and will be briefly reviewed in this chapter.

Turner syndrome, a partial or complete monosomy of the X chromosome, is a genetic disorder that occurs in 1 per 2500 live-born females. Patients may suffer from a multitude of disorders, including short stature, estrogen deficiency, infertility, and a “webbed neck.” Cardiovascular features of Turner syndrome include bicuspid aortic valve, coarctation of the aorta, hypertension, aortic dilatation, and aortic dissection. Given the disease’s multisystem involvement, care for Turner syndrome patients is often provided by a multidisciplinary team in a tertiary center. This chapter presents the various cardiovascular manifestations that may occur in patients with Turner syndrome and discusses the latest insights into the syndrome’s genetic aspects.

Pathology of sudden death and clinico-pathologic correlations are treated in this Chapter. Definition, epidemiology, and pathogenetic mechanisms are first addressed. Sudden death is mostly cardiovascular and arrhythmic in origin, with ventricular fibrillation as the precipitating mechanism of cardiac arrest. The majority of cases present with a pathologic substrate at the level of coronary arteries, myocardium, valves, conduction system, and great arteries. However, there are cases without substrate (ion channel diseases) which require molecular investigations in search of transmissible disease. The chapter is mostly devoted to sudden death in the young, with particular reference to the athletes. Anatomy and pathology of the cardiac conduction system is also dealt with, including ECG correlations; AV block and ventricular pre-excitation are taken into detailed consideration. Atrial fibrillation, its causes, substrates, potential complications, and treatment are also extensively treated. Guidelines and useful recommendations for postmortem study of sudden death appear at the end of the chapter.

Identifying the cause of sudden unexpected cardiac death requires not only examination of the heart but also a complete autopsy with toxicology analysis, thorough scene investigation, including activities and behaviors at the time of unresponsiveness, and review of the medical history for the decedent as well as the family. Documentation of pathologic changes as well as artifacts of medical intervention and postmortem changes will allow for accurate determination of cause of death. In cases where gross and microscopic examination are normal and circumstances, history, and toxicology do not explain death, genetic analysis particularly for cardiac channelopathies but also for other alterations may elucidate the cause of sudden cardiac death. Consultation with a cardiac pathologist will allow for thorough examination and documentation in complex cases where the expertise will aid in understanding of pathologic processes and the extent of disease.

Rupture of isolated iliac artery dissections is a life-threatening event and has not been associated with cystic medial degeneration (CMD) Erdheim–Gsell.

A young man presented to our emergency unit in a critical hemodynamic condition. Computed tomography scan feigned the rupture of an internal iliac artery.

Surprisingly, we found a ruptured dissection of the common iliac artery during open surgery. Reconstruction was done by Dacron graft interposition between common and internal iliac artery and external iliac artery implantation into the graft. Histology revealed CMD.

Spontaneous iliac artery dissection and rupture can be caused by CMD and requires immediate surgical intervention.