, Domenico Corrado2 and Cristina Basso1
(1)
Cardiovascular Pathology Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy
(2)
Cardiology Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy
Since SD, especially in the young, is frequently the first and last symptom of an underlying disease, the autopsy is the only way to establish the cause. Thus, it should be regularly carried out, not only for the right of the family and community to have an explanation of this dramatic event but also for the implications on the surviving relatives [1]. About 30–40 % of SDs are indeed ascribable to genetically determined, transmissible morbid entities, mostly with an autosomal dominant pattern of inheritance, so that 50 % of the first-degree relatives are potentially affected (“carriers”) of the mutation and exposed at risk [1–5].
As emphasized in the guidelines of the Association for European Cardiovascular Pathology, the role of the autopsy is to establish [1]:
(a)
Whether the death is attributable to natural or unnatural causes and, if natural, to a cardiac or extracardiac disease
(b)
The nature of the cardiac disease and whether the mechanism was arrhythmic or mechanical
(c)
Whether the cardiac condition, causing SD, may be inherited, requiring screening and counseling of the next of kin
(d)
If toxic or illicit drug abuse is involved and if other unnatural deaths played a role
10.1 Clinical Information Relevant to Autopsy
Gathering the relevant clinical information is mandatory for a clinicopathologic correlation [1]:
Age, gender, occupation, and lifestyle (alcohol, tabagism, etc.)
Circumstances of death: witnessed or unwitnessed, any suspicious circumstances (carbon monoxide, violence, traffic accident, electrical burst, etc.), and ECG tracing recorded during resuscitation
Family history, especially cardiac: ischemic heart disease, premature SD, arrhythmias, and inherited arrhythmic disorders
Medical history: general health status, previous significant illnesses (syncope, epilepsy, chest pain, palpitations particularly during exercise, etc.), previous surgical or nonsurgical interventions (pacemaker, implantable cardioverter defibrillator-ICD, etc.), ECG tracings and chest X-rays, echo, and any laboratory findings (lipid profiles, serum enzymes and troponin measurements, medication, etc.)
10.2 External Examination of the Body
Establish body weight and height (to correlate with heart weight and wall thickness).
Check for recent intravenous access, intubation, ECG pads, defibrillator and electrical burns, drain sites, and traumatic lesions.
Check for ICD/pacemaker; if in situ, see MDA Safety Notice 2002 for safe removal and interrogation.
10.3 Sequential Approach to Establish the Causes of Sudden Death through Full Autopsy Technique
Noncardiac causes of sudden death should be first excluded [1]. Any natural sudden death can be considered cardiac in origin after the exclusion of noncardiac causes. Thus, a full autopsy with sequential approach should be always performed to rule out common and uncommon extracardiac causes of SD:
Cerebral (subarachnoid or intracerebral hemorrhage, etc.)
Respiratory (allergic, asthma, anaphylaxis, etc.)
Acute hemorrhagic shock (ruptured aortic aneurysm, gastrointestinal hemorrhage, etc.)
Septic shock (Waterhouse–Friderichsen syndrome, etc.)
10.4 Search for the Cardiac Culprit: The Standard Gross Examination
The search for the cardiac culprit should address the vital components of the heart [1]: great arteries, coronary arteries, myocardium, valves, and conduction system.
The standard gross examination of the heart should be carried out as follows:
2.
Check the great arteries before transecting them, 3 cm above the aortic and pulmonary valves, and open the pulmonary artery and bifurcation (Fig. 10.2).
3.
Check and transect the pulmonary veins. Transect the superior vena cava 2 cm above the point where the crista terminalis meets the superior vena cava (sulcus terminalis), to preserve the sinus node. Transect the inferior vena cava close to the diaphragm.
4.
Open the right atrium from the inferior vena cava to the apex of the right atrial appendage. Open the left atrium between the pulmonary veins and then to the atrial appendage (Fig. 10.3).
5.
Inspect the atrial cavities and the interatrial septum, and determine whether the foramen ovale is patent. Examine the mitral and tricuspid valves (or valve prostheses) and check the integrity of the papillary muscles and chordae tendineae.
6.
Inspect the aorta, the pulmonary artery, and the aortic and pulmonary valves (or valve prosthesis).
7.
Then the coronary arteries are grossly investigated as follows:
(b)
Assess the size, course, and “dominance” of the major epicardial arteries.
(c)
(d)
Heavily calcified coronary arteries have sometimes to be opened adequately with sharp scissors. If this is not possible, they should be removed intact, decalcified, and opened transversely.
(e)
Coronary artery segments containing metallic stents should be referred intact to labs with facilities for resin embedding and subsequent processing and sectioning.
(f)
Coronary artery bypass grafts (saphenous veins, internal mammary arteries, radial arteries, etc.) should be carefully examined with transverse cuts. The proximal and distal anastomoses should be examined with particular care.
8.
Make a complete transverse (short-axis) cut of the heart at the midventricular level and then parallel slices of ventricles at 1 cm intervals toward the apex (Fig. 10.7), and assess these slices carefully for thickness of the walls and size of the cavities, as well as abnormalities of the myocardium.
9.
Once emptied of blood, make the following measurements:
Total heart weight (against tables of normal weights by age, gender, and body weight).
Heart wall thickness: inspect the endocardium and measure thickness of the mid-cavity free wall of the left ventricle, right ventricle, and septum (excluding trabeculae), against tables of normal thickness by age, gender, and body weight.
Heart dimensions: the transverse size is best calculated as the distance from the obtuse to the acute margin in the posterior AV sulcus. The longitudinal size is obtained from a measurement of the distance between the crux cordis and the apex of the heart on the posterior aspect.
10.
Open the basal half of the heart along the blood flow direction and complete the examination of atrial and ventricular septa, AV valves, ventricular inflows and outflows, and semilunar valves (Fig. 10.8). In case of ECG-documented ventricular preexcitation, the AV rings should be maintained intact.
10.5 Standard Histological Examination of the Heart
Coronary arteries: in the setting of coronary artery disease, the severe focal lesions should be sampled for histology in labeled blocks and stained routinely with hematoxylin-eosin and a connective tissue stain (elastic Weigert–van Gieson, trichrome, or Sirius red). Special stains and immunohistochemistry should be performed as deemed necessary.< div class='tao-gold-member'>Only gold members can continue reading. Log In or Register to continue
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