CARDIOVASCULAR DISORDERS

RADIOLOGIC PATHOLOGY LECTURE BY: ELOISA D. DE LUNA, M.D., M.P.H.

PRINCIPLES OF CARDIAC DYSFUNCTION

MECHANISMS: 1. LOSS OF BLOOD 2. IRREGULAR HEARTBEAT 3. OBSTRUCTED FLOW 4. REGURGITANT OR MISDIRECTED FLOW 5. PUMP FAILURE
SYSTOLIC FAILURE CONTRACTILE DYSFUNCTION DIASTOLIC FAILURE INADEQUATE FILLING

PRINCIPLES OF CARDIAC DYSFUNCTION

1. LOSS OF BLOOD DISRUPTION OF THE CONTINUITY OF THE CIRCULATORY SYSTEM (E.G., GUNSHOT WOUND THROUGH THE THORACIC AORTA) THAT PERMITS BLOOD TO ESCAPE (HEART CANNOT FILL, AND THE RESISTANCE AGAINST WHICH IT PUMPS IS LOST

PRINCIPLES OF CARDIAC DYSFUNCTION

2. IRREGULAR HEARTBEAT DISORDERS OF CARDIAC CONDUCTION (E.G. HEART BLOCK) OR ARRHYTHMIAS OWING TO GENERATION OF IMPULSES IN AN UNCOORDINATED MANNER (E.G., VENTRICULAR FIBRILLATION) LEADING TO CONTRACTIONS OF THE MUSCULAR WALLS THAT ARE NOT UNIFORM AND EFFICIENT

PRINCIPLES OF CARDIAC DYSFUNCTION

3. OBSTRUCTED FLOW A LESION PREVENTING VALVE OPENING OR ONE NARROWING THE LUMEN OF A VESSEL (E.G., AORTIC VALVULAR STENOSIS OR COARCTATION) OBSTRUCTING BLOOD FLOW AND OVERWORKS THE PUMP BEHIND THE OBSTRUCTION

PRINCIPLES OF CARDIAC DYSFUNCTION

4. REGURGITANT OR MISDIRECTED FLOW (E.G., MITRAL OR AORTIC VALVULAR REGURGITATION) CAUSING SOME OF THE OUTPUT FROM EACH CONTRACTION TO REFLUX BACKWARD, NECESSARILY FORCING PORTIONS OF THE PUMP TO EXPEL THE SAME BLOOD SEVERAL TIMES, THUS INDUCING SUBSTANTIAL MYOCARDIAL STRESS

PRINCIPLES OF CARDIAC DYSFUNCTION

5. FAILURE OF THE PUMP ITSELF DAMAGED MUSCLE ITSELF CONTRACTS WEAKLY OR INADEQUATELY, AND THE CHAMBERS CANNOT EMPTY PROPERLY. - THE MUSCLE CANNOT RELAX SUFFICIENTLY, THE LEFT VENTRICULAR CHAMBER CANNOT DILATE DURING DIASTOLE AND THE HEART CANNOT PROPERLY FILL

CONGESTIVE HEART FAILURE

ANY ONE OF THE CAUSES ABOVE MENTIONED, WHEN SUFFICIENTLY SEVERE OR ADVANCED, ULTIMATELY IMPAIRS CARDIAC FUNCTION AND RENDER THE HEART UNABLE TO MAINTAIN AN OUTPUT SUFFICIENT FOR THE METABOLIC REQUIREMENTS OF THE TISSUES AND ORGANS OF THE BODY, PRODUCING CONGESTIVE HEART FAILURE (CHF)

CHF
CHARACTERIZED BY: 1. DIMINISHED CARDIAC OUTPUT 2. DAMNING BACK OF BLOOD IN THE VENOUS SYSTEM 3. BOTH

CHF
OCCURS: 1. DUE TO A DECREASED MYOCARDIAL CAPACITY TO CONTRACT
THERE IS PROGRESSIVE DETERIORATION OF MYOCARDIAL CONTRACTILITY FUNCTION (SYSTOLIC DYSFUNCTION)
OCCURS IN : (ISCHEMIC INJURY, PRESSURE OR VOLUME OVERLOAD, DILATED CARDIOMYOPATHY)

CHF
2. DUE TO AN INABILITY TO FILL THE CARDIAC CHAMBERS WITH BLOOD - INABILITY OF THE CARDIAC CHAMBERS TO EXPAND SUFFICIENTLY DURING DIASTOLE TO ACCOMMODATE AN ADEQUATE VENTRICULAR BLOOD VOLUMES (DIASTOLIC DYSFUNCTION) OCCURS IN: MASSIVE LEFT VENTRICULAR HYPERTROPHY, MYOCARDIAL FIBROSIS, DEPOSITION OF AMYLOID,CONSTRICTIVE PERICARDITIS

CARDIAC HYPERTROPHY
COMPENSATORY RESPONSE OF THE MYOCARDIUM TO INCREASED WORK: PATTERNS OF HYPERTROPHY DEPENDING ON STIMULUS: 1. PRESSURE-OVERLOAD VENTRICLES (HPN OR AORTIC STENOSIS) DEVELOP CONCENTRIC HYPERTROPHY: INCREASED RATIO OF WALL THICKNESS TO CAVITY

CARDIAC HYPERTROPHY
PATTERNS (CONTD.) 2. VOLUME-OVERLOAD VENTRICLES (MITRAL REGURGITATION) DEVELOP HYPERTROPHY WITH DILATATION (ECCENTRIC HYPERTROPHY) WITH PROPORTIONATE INCREASES IN VENTRICULAR RADIUS AND WALL THICKNESS

TYPES OF HEART DISEASE

1. 2. 3. 4. 5. 6. ISCHEMIC HEART DISEASE CARDIOMYOPATHIES PULMONARY VASCULAR DISEASE ACQUIRED VALVULAR DISEASE CARDIAC MASSES PERICARDIAL DISEASE

ISCHEMIC HEART DISEASE (IHD)

CORONARY HEART DISEASE (CHD)

CLINICAL PRESENTATIONS: 1. STABLE ANGINA 2. UNSTABLE ANGINA (PREINFARCTION) 3. ACUTE MYOCARDIAL INFARCTION 4. CHF SECONDARY TO CHRONIC ISCHEMIA 5. ARRHYTHMIAS 6. SUDDEN DEATH

IHD
RISK FACTORS FOR DEVELOPMENT OF ATHEROSCLEROTIC CORONARY ARTERY DISEASE: 1. INCREASED LEVELS OF SERUM CHOLESTEROL 2. TOBACCO SMOKING 3. DIABETES 4. HPN 5. SEDENTARY LIFE STYLE 6. AGE 7. MALE GENDER 8. HEREDITY

IHD
CLINICAL SYMPTOMS CAUSED BY LUMINAL ABNORMALITIES OF THE CORONARY ARTERIES INCLUDING: A. ATHEROMATOUS DISEASE B. CORONARY THROMBOSIS C. INTRALUMINAL ULCERATION AND HEMORRHAGE D. VASOCONSTRICTION E. CORONARY ECTASIA AND ANEURYSM

IHD
IMAGING WORKUP: 1. CHEST RADIOGRAPHY 2. NUCLEAR MEDICINE PERFUSION SCAN 3. CORONARY ANGIOGRAPHY

IHD
CORONARY ARTERY CALCIFICATION OCCURS IN THE INTIMA - DIRECTLY RELATED TO ADVANCED ATHEROMATOUS DISEASE AND CORONARY NARROWING - DETECTION HAS A PREDICTIVE ACCURACY OF 86% (IN ASYMPTOMATIC POPULATION)

MYOCARDIAL INFARCTION
ACUTE MI NORMAL CXR (90%) CARDIOMEGALY AND CONGESTIVE FAILURE (60-70%) ANTERIOR WALL INFARCTION MULTIVESSEL DISEASE LEFT VENTRICLE ANEURYSM

MYOCARDIAL INFARCTION
IRREVERSIBLE NECROSIS OF HEART MUSCLE SECONDARY TO PROLONGED ISCHEMIA USUALLY RESULTS FROM AN IMBALANCE OF OXYGEN SUPPLY AND DEMAND

APPEARANCE OF CARDIAC ENZYMES IN THE CIRCULATION GENERALLY INDICATES MYOCARDIAL NECROSIS

MAY LEAD TO IMPAIRMENT OF SYSTOLIC FUNCTION OR DIASTOLIC FUNCTION AND TO INCREASED PREDISPOSITION TO ARRHYTHMIAS AND OTHER LONGTERM COMPLICATIONS.

MYOCARDIAL INFARCTIONPATHOPHYSIOLOGY
ATHEROSCLEROSIS IS THE DISEASE PRIMARILY RESPONSIBLE FOR MOST ACUTE CORONARY SYNDROME CASES 90% RESULT FROM AN ACUTE THROMBUS THAT OBSTRUCTS AN ATHEROSCLEROTIC CORONARY ARTERY MAJOR TRIGGER OF CORONARY THROMBOSIS: 1. PLAQUE RUPTURE 2. PLATELET ACTIVATION AND AGGREGATION 3. COAGULATION PATHWAY ACTIVATION 4. ENDOTHELIAL VASOCONSTRICTION

LEADING TO CORONARY THROMBOSIS AND OCCLUSION.

HYPERTENSIVE HEART DISEASE

RESPONSE OF THE HEART TO THE INCREASED DEMANDS INDUCED BY SYSTEMIC OR PULMONARY HYPERTENSION DIAGNOSTIC CRITERIA: 1. LEFT VENTRICULAR HYPERTROPHY (CONCENTRIC) IN THE ABSENCE OF OTHER CV PATHOLOGY 2. HISTORY OF HPN (PROLONGED BP OF 140/90 SUFFICIENTLY MAY INDUCE LV HYPERTROPHY)

HYPERTENSIVE HEART DISEASEPATHOPHYSIOLOGY

PATHOPHYSIOLOGY OF HYPERTENSIVE HEART DISEASE IS A

COMPLEX INTERPLAY OF VARIOUS HEMODYNAMIC, STRUCTURAL, NEUROENDOCRINE, CELLULAR, AND MOLECULAR FACTORS THESE FACTORS PLAY INTEGRAL ROLES IN THE DEVELOPMENT OF HYPERTENSION AND ITS COMPLICATIONS ELEVATED BP ITSELF CAN MODULATE THESE FACTORS ELEVATED BP LEADS TO ADVERSE CHANGES IN CARDIAC STRUCTURE AND FUNCTION IN 2 WAYS: 1. DIRECTLY BY INCREASED AFTERLOAD 2. INDIRECTLY BY ASSOCIATED NEUROHORMONAL AND VASCULAR CHANGES

HYPERTENSIVE HEART DISEASE

TWO-DIMENSIONAL ECHOCARDIOGRAM OF A 70-YEAR-OLD WOMAN (PARASTERNAL LONG AXIS VIEW) SHOWING CONCENTRIC LEFT VENTRICULAR HYPERTROPHY.

LEFT VENTRICULAR HYPERTROPHY

GROSS SPECIMEN OF THE HEART WITH CONCENTRIC LEFT VENTRICULAR HYPERTROPHY.

PERICARDIAL DISEASE

PERICARDIAL DISEASE
PERICARDIAL EFFUSION MOST COMMON ABNORMALITY OF THE PERICARDIUM PLAIN FILM THICKENING OF THE PERICARDIAL STRIPE (CXR/CT: 2-3 MM; MR: <4 MM) CHRONIC WATER BOTTLE CONFIGURATION NORMAL PERICARDIUM - 20 ML OF FLUID
200 ML DETECTABLE BY PLAIN FILM

PERICARDIAL EFFUSION
PRESENCE OF AN ABNORMAL AMOUNT AND/OR CHARACTER OF FLUID IN THE PERICARDIAL SPACE CAUSED BY A VARIETY OF LOCAL AND SYSTEMIC DISORDERS, OR IT MAY BE IDIOPATHIC ACUTE OR CHRONIC, AND THE TIME COURSE OF DEVELOPMENT HAS A GREAT IMPACT ON THE PATIENT'S SYMPTOMS

PERICARDIAL EFFUSION
ECHOCARDIOGRAPHY DETECTS:
<50ML (APPEARS AS POSTERIOR SONOLUCENT COLLECTION) <100 ML (SMALL) ANTERIOR AND POSTERIOR SONOLUCENT REGIONS 100-500 ML (MODERATE) SONOLUCENT ZONE AROUND THE ENTIRE VENTRICLE >500 ML (VERY LARGE) EXTENDS BEYOND THE FIELD OF VIEW

PERICARDIAL EFFUSION IMAGE IS FROM A PATIENT WITH MALIGNANT PERICARDIAL EFFUSION. NOTE THE "WATERBOTTLE" APPEARANCE OF THE CARDIAC SILHOUETTE IN THE ANTEROPOSTERIOR (AP) CHEST FILM.

CARDIAC TAMPONADE
A CLINICAL SYNDROME CAUSED BY THE ACCUMULATION OF FLUID IN THE PERICARDIAL SPACE RESULTING IN REDUCED VENTRICULAR FILLING AND SUBSEQUENT HEMODYNAMIC COMPROMISE. CARDIAC TAMPONADE IS A MEDICAL EMERGENCY. THE OVERALL RISK OF DEATH DEPENDS ON THE SPEED OF DIAGNOSIS, THE TREATMENT PROVIDED, AND THE UNDERLYING CAUSE OF THE TAMPONADE.

CARDIAC TAMPONADE
CARDIAC CHAMBER COMPRESSION BY PERICARDIAL EFFUSION UNDER TENSION, COMPROMISING DIASTOLIC FILLING CLINICAL EXAM:
MARKED JUGULAR VENOUS DISTENSION DISTANT HEART SOUNDS PERICARDIAL RUB CXR: RAPID ENLARGEMENT OF THE CARDIAC SILHOUTTE, NORMAL APPEARING VASCULARITY

CARDIAC TAMPONADE

THIS ANTEROPOSTERIORVIEW CHEST RADIOGRAPH SHOWS A MASSIVE BOTTLESHAPED HEART AND CONSPICUOUS ABSENCE OF PULMONARY VASCULAR CONGESTION. REPRODUCED WITH PERMISSION FROM CHEST, 1996: 109:825.

TRAUMA

AORTA, TRAUMA ANGIOGRAM SHOWS A FOCAL ISTHMUS TRAUMATIC PSEUDOANEURYSM. THIS EXAMPLE DEMONSTRATES AN ANTEROMEDIAL BULGE, INTIMAL FLAP, ACUTE-ANGLE TRANSITION, IRREGULAR CONTOUR, AND CONTRAST-MATERIAL HANG UP IN THIS 45-YEAROLD FEMALE PATIENT.

AORTA, TRAUMA CHEST RADIOGRAPH SHOWS WIDENING OF THE MEDIASTINAL CONTOUR AND DEFORMITY AND BLURRED MARGINS OF THE SUPERIOR MEDIASTINUM.

CARDIOMYOPATHIES
GROUP OF ANOMALIES WITH 3 BASIC FEATURES: 1. FAILURE OF THE HEART TO MAINTAIN ITS ARCHITECTURE 2. FAILURE OF THE HEART TO MAINTAIN NORMAL ELECTRICAL ACTIVITY 3. FAILURE OF THE HEART TO MAINTAIN CARDIAC OUTPUT

CARDIOMYOPATHIES
GENERAL FEATURES: 1. CARDIOMEGALY 2. CHF (OFTEN, LUNGS ARE CLEAR) 3. DILATED LEFT AND RIGHT VENTRICLES WITH ELEVATED END-DIASTOLIC PRESSURES AND DECREASED CONTRACTILITY 4. DECREASED EJECTION FRACTIONS

CARDIOMYOPATHIES
TYPES: 1. DILATED C. GLOBAL CARDIOMEGALY 2. HYPERTROPHIC C. FAMILIAL, AD, ASSOCIATED WITH NEUROFIBROMATOSIS, NOONANS SYNDROME, SECONDARY TO PRESSURE OVERLOAD (ANGINA, SYNCOPE, ARRYTHMIAS, CHF, SUDDEN DEATH

DILATED CARDIOMYOPATHY MAGNIFIED CHEST RADIOGRAPH OF THE RIGHT COSTOPHRENIC ANGLE IN A PATIENT WITH DILATED CARDIOMYOPATHY AND INTERSTITIAL PULMONARY EDEMA, AS DEMONSTRATED BY PROMINENT SEPTAL LINES (KERLEY B LINES). THE LINES ARE USUALLY HORIZONTAL AND SEEN IN THE PERIPHERAL PARTS OF THE LUNGS SECONDARY TO INTERSTITIAL EDEMA.

DILATED CARDIOMYOPATHY CHEST RADIOGRAPH SHOWS A LARGE HEART. IMAGE DOES NOT PROVIDE ANY CLUE TO THE ETIOLOGY OF THE DILATED CARDIOMYOPATHY; HOWEVER, THE HEALING RIB FRACTURES AND RIGHT LOWER LOBE PNEUMONIA (ASPIRATION PNEUMONIA) SUGGEST ALCOHOLIC CARDIOMYOPATHY AS THE CAUSE.

CARDIOMYOPATHIES
3. RESTRICTIVE C. S/S RELATED TO CHF (PATHOPHYSIOLOGY: IMPAIRED DIASTOLIC FUNCTION WITH DECREASED VENTRICULAR COMPLIANCE, POOR DIASTOLIC FILLING AND ELEVATION OF RIGHT AND LEFT VENTRICULAR FILLING PRESSURES 4. UHLS ANOMALY RARE, DILATION OF THE RV WITH MARKED THINNING OF THE ANTERIOR RIGHT VENTRICULAR WALL

RESTRICTIVE CARDIOMYOPATHY

MRI OF THE HEART IN A 30-YEAR-OLD WOMAN WITH SARCOID DEMONSTRATES A NORMAL PERICARDIUM.

RESTRICTIVE CARDIOMYOPATHY AXIAL CONTRASTENHANCED CT IMAGE THROUGH THE HEART SHOWS A THIN PERICARDIUM WITHOUT CALCIFICATION. NOTE THE CARDIOPHRENIC AND INTERNAL MAMMARY LYMPH NODES. THE PATIENT HAD EXTENSIVE MEDIASTINAL AND HILAR ADENOPATHY, AS WELL AS INTERSTITIAL LUNG CHANGES.

ACQUIRED VALVULAR HEART DISEASE

MITRAL STENOSIS CAUSED BY RHEUMATIC HEART DISEASE NORMAL MITRAL VALVE AREA: 4-6 CM2 MILD MITRAL S. (<1.5 CM2) CXR NORMAL S/S: DYSPNEA ON EXERTION

MITRAL STENOSIS
SEVERE MITRAL S. (<0.5 CM2) - MARKED LEFT ATRIAL ENLARGEMENT, RIGHT VENTRICULAR ENLARGEMENT , KERLEYS LINES, PULMONARY EDEMA, CALCIFICATION IN LEFT ATRIAL WALL, DYSPNEA AT REST

MITRAL STENOSIS Rheumatic mitral stenosis with commissural fusion and enlarged left atrium. Calcification of the subvalvular structures is minimal and has a low Massachusetts General Hospital (MGH) score; this condition is potentially amenable to balloon valvotomy.

AORTIC STENOSIS
CAUSE: PARTIAL FUSION OF THE COMMISSURES BETWEEN THE AORTIC VALVE CUSPS BICUSPID AORTIC VALVE :
(+) 95% OF PATIENTS WITH CONGENITAL AORTIC STENOSIS (+) 25-50% OF PATIENTS WITH COARCTATION OF THE AORTA CALCIFIC OR DEGENERATIVE AORTIC STENOSIS OLDER PATIENTS WITH SYSTEMIC HYPERTENSION

AORTIC STENOSIS
NORMAL AORTIC VALVE ORIFICE: 3CM2 AORTIC STENOSIS: <0.7CM2
OR <1.5CM2 ((+) AORTIC STENOSIS AND INSUFFICIENCY) CXR: LEFT VENTRICULAR HYPERTROPHY WITH POSTSTENOTIC DILATATION OF THE AORTA ECHOCARDIOGRAM: DENSE AORTIC VALVE ECHOES, DILATED AORTIC ROOT, HYPERDYNAMIC FUNCTION AND LEFT VENTRICULAR HYPERTROPHY

AORTIC STENOSIS

VALVULAR CALCIFICATION OF AORTIC STENOSIS SEEN WITH CARDIAC FLUOROSCOPY DURING CATHETERIZATION.

AORTIC REGURGITATION
AFFECTS 10% OF ALL PATIENTS WITH VALVULAR HEART DISEASE CHARACTERIZED BY :
AN ABNORMAL BACKWARD LEAKAGE OF BLOOD FROM THE AORTA INTO THE LEFT VENTRICLE (LV) DURING THE DIASTOLIC PHASE OF THE CARDIAC CYCLE

THE DISEASE PROCESS MAY INVOLVE THE AORTIC VALVE CUSPS, THE AORTIC ROOT, OR BOTH.1,2

AORTIC REGURGITATION
CLINICAL MANIFESTATION : - RECOGNIZED IN ASSOCIATION WITH AN ASYMPTOMATIC HEART MURMUR ON PHYSICAL EXAMINATION, OR - SYMPTOMS OF SUBSTANTIAL LV DYSFUNCTION AND HEART FAILURE DIAGNOSIS: ECHOCARDIOGRAPHY - MOST IMPORTANT IMAGING MODALITY - DETERMINE CAUSES AND SEVERITY AND TREATMENT

AORTIC REGURGITATION
TREATMENT OFTEN DEPENDS ON WHETHER THE REGURGITATION DEVELOPS ACUTELY OR AS A CHRONIC DISEASE. - ACUTE AORTIC REGURGITATION - POOR PROGNOSIS UNLESS RAPID SURGICAL TREATMENT IS UNDERTAKEN. - CHRONIC AORTIC REGURGITATION MAY REMAIN ASYMPTOMATIC FOR YEARS. - PROGNOSIS FOR ASYMPTOMATIC PATIENTS IS GENERALLY GOOD, AS COMPARED WITH PATIENTS WHO HAVE SYMPTOMS OF LV DYSFUNCTION.

AORTIC REGURGITATION

Aortic regurgitation. Chest radiograph in a patient with aortic dissection and acute aortic regurgitation shows a cardiac silhouette of essentially normal dimension.

AORTIC REGURGITATION

Aortic-root angiography shows regurgitation of contrast material into the left ventricle (LV).

CARDIAC MASSES
INCLUDE THROMBI, PRIMARY BENIGN TUMORS, PRIMARY MALIGNANT TUMORS AND METASTATIC TUMORS MOST DO NOT DEFORM THE OUTER CONTOURS OF THE HEART IDENTIFIED BY NUCLEAR SCINTIGRAPHY, CT AND CARDIAC ANGIOGRAPHY

CARDIAC MASSES
THROMBI - MOST FREQUENT CAUSE OF AN INTRACARDIAC MASS MOST COMMON IN THE LA AND LV AND PRESENT WITH A RISK OF SYSTEMIC EMBOLI OCCURS ALONG THE POSTERIOR WALL OF THE LA DIFFERENTIATE TUMOR VS CLOT DONE WITH MR:
CLOTS- LOW SIGNAL TUMOR INTERMEDIATE SIGNAL

CARDIAC MASSES - BENIGN

ATRIAL MYXOMA 50% OF CARDIAC TUMORS - MOST COMMON BENIGN PRIMARY TUMOR 30-60 YEARS OF AGE ACCOMPANIED BY FEVER, ANEMIA, WEIGHT LOSS, EMBOLIC SYMPTOMS OR SYNCOPE

ATRIAL MYXOMA

POSTEROANTERIOR (PA) CHEST RADIOGRAPH SHOWS PULMONARY VENOUS CONGESTION AND PROMINENCE OF THE LEFT ATRIAL APPENDAGE IN A PATIENT WITH A LEFT ATRIAL MYXOMA OBSTRUCTING THE MITRAL VALVE.

ATRIAL MYXOMA T1-WEIGHTED TURBO SPIN-ECHO MRI SCAN SHOWS INTERMEDIATE SIGNAL INTENSITY IN A LEFT ATRIAL MYXOMA (M) ATTACHED TO THE MARGIN OF THE FOSSA OVALIS (BETWEEN THE ARROWS). LA INDICATES THE LEFT ATRIUM; RA, THE RIGHT ATRIUM; AND RV, THE RIGHT VENTRICLE.

CARDIAC MASSES - MALIGNANT

METASTATIC CARDIAC TUMORS MORE COMMON THAN PRIMARY CARDIAC TUMORS FROM: BREAST, LUNG, MELANOMA AND LYMPHOMA MR EXCELLENT FOR DETECTING DIRECT EXTENSION, INTRACARDIAC METS AND PERICARDIAL INVOLVEMENT ANGIOSARCOMA MOST COMMON PRIMARY MALIGNANT CARDIAC TUMOR (RHABDOSARCOMA, LIPOSARCOMA)

ANGIOSARCOMA Angiosarcoma of the heart, with pericardial encasement by hemorrhagic tumor. Image courtesy of Dr. K. Marchant.

CONGENITAL HEART DISEASE

ABNORMALITIES OF THE HEART OR GREAT VESSELS THAT ARE PRESENT FROM BIRTH ARISE FROM FAULTY EMBRYOGENESIS, DURING GESTATIONAL WEEKS 3-8 WEEKS, PERIOD WHEN MAJOR CARDIOVASCULAR STRUCTURES DEVELOP ETIOLOGY: 90% UNKNOWN MULTIFACTORIAL GENETIC (CHROMOSOMAL DEFECTS)AND ENVIRONMENTAL INPUTS (VIRUSES, CHEMICALS, RADIATION)

CONGENITAL HEART DISEASE

TWO MAJOR CATEGORIES: 1. SHUNT ABNORMAL COMMUNICATION BETWEEN CHAMBERS OR BLOOD VESSELS OR BOTH 2. OBSTRUCTION SHUNTS - ABNORMAL CHANNELS PERMIT THE FLOW OF BLOOD FROM LEFT TO RIGHT OR THE REVERSE, DEPENDING ON PRESSURE RELATIONSHIPS

CONGENITAL HEART DISEASE

RIGHT-TO-LEFT SHUNT A DUSKY BLUENESS OF THE SKIN AND MUCOUS MEMBRANES (CYANOSIS) RESULTS BECAUSE OF POORLY OXYGENATED BLOOD ENTERS THE SYSTEMIC CIRCULATION (E.G., TETRALOGY OF FALLOT, TRANSPOSITION OF THE GREAT ARTERIES (TGA), PERSISTENT TRUNCUS ARTERIOSUS, TRICUSPID ATRESIA AND TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION.

CONGENITAL HEART DISEASE

LEFT-TO-RIGHT SHUNT (-LATE CYANOSIS) NOT INITIALLY ASSOCIATED WITH CYANOSIS, BUT CAN RESULT IN PROGRESSIVE PULMONARY HYPERTENSION AND RIGHT VENTRICULAR OVERLOAD WITH HYPERTROPHY MUSCULAR PULMONARY ARTERIES FIRST RESPOND TOINCREASED PRESSURE BY MEDIAL HYPERTROPHY AND VASOCONSTRICTION TO MAINTAIN NORMAL DISTAL PULMONARY CAPILLARY AND VENOUS PRESSURE AND HELP PREVENT PULMONARY EDEMA.

CONGENITAL HEART DISEASE

LEFT-TO-RIGHT SHUNTS (CONTD.) PROLONGED PULMONARY ARTERIAL VASOCONSTRICTION STIMULATES DEVELOPMENT OF IRREVERSIBLE OBSTRUCTIVE INTIMAL LESIONS LEADING TO PRESSURE ON THE RIGHT SIDE OF THE HEART TO RISE EXCEEDING THAT ON THE LEFT, EVENTUALLY REVERSING THE SHUNT TO RIGHT-TO-LEFT SHUNT (E.G. ATRIAL SEPTAL DEFECT, VENTRICULAR SEPTAL DEFECT, PATENT DUCTUS ARTERIOSUS AND ATRIOVENTRICULAR SEPTAL DEFECT)

RIGHT TO LEFT SHUNT EARLY CYANOSIS TETRALOGY OF FALLOT

MOST COMMON TYPE OF CYANOTIC CONGENITAL HEART DISEASE CONSISTS : 1. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION 2. A MALALIGNMENT VENTRICULAR SEPTAL DEFECT 3. AN OVERRIDING AORTA 4. RIGHT VENTRICULAR HYPERTROPHY

TETRALOGY OF FALLOT PATHOPHYSIOLOGY

1. ANTERIOR AND SUPERIOR DISPLACEMENT OF THE INFUNDIBULAR SEPTUM 2. CAUSES NARROWING OF THE DEVELOPING RIGHT VENTRICULAR OUTFLOW TRACT 3. A VENTRICULAR SEPTAL DEFECT AND AN OVERRIDING AORTA ARE THE CONSEQUENCES OF THE SEPTAL DISPLACEMENT. 4. THE LARGE VENTRICULAR SEPTAL DEFECT EQUALIZES THE RIGHT AND LEFT VENTRICULAR PRESSURES, RESULTING IN PERSISTENT RIGHT VENTRICULAR HYPERTROPHY RIGHT-TO-LEFT SHUNTING VARIES WITH THE DEGREE OF PULMONARY OUTFLOW TRACT OBSTRUCTION, BUT IT IS PROGRESSIVE.

TETRALOGY OF FALLOT INFANT WITH TETRALOGY OF FALLOT AND A RIGHTSIDED AORTIC ARCH

TETRALOGY OF FALLOT

CHEST RADIOGRAPH OF BOOT-SHAPED HEART IN AN INFANT WITH TETRALOGY OF FALLOT

TETRALOGY OF FALLOT 1. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION 2. A MALALIGNMENT VENTRICULAR SEPTAL DEFECT 3. AN OVERRIDING AORTA 4. RIGHT VENTRICULAR HYPERTROPHY

TETRALOGY OF FALLOT
1. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION 2. A MALALIGNMENT VENTRICULAR SEPTAL DEFECT 3. AN OVERRIDING AORTA 4. RIGHT VENTRICULAR HYPERTROPHY

EBSTEIN ANOMALY
CONGENITAL MALFORMATION OF THE HEART CHARACTERIZED BY: 1. APICAL DISPLACEMENT OF THE SEPTAL AND POSTERIOR TRICUSPID VALVE LEAFLETS, LEADING TO 2. ATRIALIZATION OF THE RIGHT VENTRICLE 3. WITH A VARIABLE DEGREE OF MALFORMATION AND DISPLACEMENT OF THE ANTERIOR LEAFLET.

EBSTEIN ANOMALY
SOME OF THE ABNORMALITIES ASSOCIATED WITH EBSTEIN ANOMALY: VENTRICULAR SEPTAL DEFECT (VSD) AORTIC COARCTATION INTERATRIAL COMMUNICATION - ASD, ESPECIALLY SECUNDUM ASD, AND PATENT FORAMEN OVALE PULMONARY ATRESIA WITH AN INTACT VENTRICULAR SEPTUM PARTIAL ATRIOVENTRICULAR CANAL HYPOPLASTIC PULMONARY ARTERIES

ETIOLOGY
UNCERTAIN CAUSE ENVIRONMENTAL FACTORS: MATERNAL INGESTION OF LITHIUM IN FIRST TRIMESTER OF PREGNANCY MATERNAL BENZODIAZEPINE* USE MATERNAL EXPOSURE TO VARNISHING SUBSTANCES MATERNAL HISTORY OF PREVIOUS FETAL LOSS RISK IS HIGHER IN WHITES THAN IN OTHER RACES.

FRONTAL CHEST RADIOGRAPH IN AN INFANT WITH SEVERE EBSTEIN ANOMALY SHOWS A LARGE HEART THAT LEAVES LITTLE SPACE FOR THE LUNG. ALTHOUGH THE APPEARANCE IS RELATIVELY NONSPECIFIC, THE LARGE HEART SHOULD SUGGEST EBSTEIN ANOMALY IN THE DIFFERENTIAL DIAGNOSIS.

FRONTAL CHEST RADIOGRAPH IN AN ADULT WITH EBSTEIN ANOMALY SHOWS

THE ABNORMAL CONTOUR ASSOCIATED WITH DISTORTION OF THE RIGHT VENTRICLE. THE PULMONARY VASCULATURE IS UNDERPERFUSED.

LATERAL CHEST RADIOGRAPH IN AN ADULT WITH EBSTEIN ANOMALY (SAME PATIENT AS IN IMAGE 2) SHOWS PROMINENT FILLING OF THE RETROSTERNAL SPACE (ARROW) RELATED TO DISTORTION OF THE RIGHT VENTRICLE.

Media file 5: Axial ECG-gated spin-echo MRI in a patient with Ebstein anomaly (same patient as in Image 4) shows enlargement of the right atrium (RA). The attachment of the septal leaflet (arrow) of the tricuspid valve is seen displaced toward the apex of the right ventricle (RV). LV = left ventricle.

MRI IN A PATIENT WITH EBSTEIN ANOMALY SHOWS ENLARGEMENT OF THE RIGHT ATRIUM (RA). THE ATTACHMENT OF THE SEPTAL LEAFLET (ARROW) OF THE TRICUSPID VALVE IS SEEN DISPLACED TOWARD THE APEX OF THE RIGHT VENTRICLE (RV). LV = LEFT VENTRICLE.

REFERENCES
COTRAN, KUMAR, ROBBINS, PATHOLOGIC BASIS OF DISEASE 5TH ED., W.B. SAUNDERS COMPANY, 1994 BRANT W. AND HELMS C., FUNDAMENTALS OF DIAGNOSTIC RADIOLOGY, 2ND ED., LIPPINCOTT WILLIAMS & WILKINS, 1999