Cyanotic Congenital Heart Diseases, Etc.

CYANOTIC CONGENITAL HEART DISEASES 1. Ventricular Septal Defect 2. Atrial Septal Defect 3. Patent Ductus Arteriosus CASE 1: Ventricular Septal Defect 1 month old boy; Failure to thrive; CHF PE: Systolic murmur-low pericardium Holosystic murmur –extends 2nd heart sound Soft low pitch early diastolic murmur – apical area CXR FINDINGS: • Cardiomegaly • Inc pulmo bld flow • LAE • Displacement of mainstem bronchus • R aortic arch

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• CHF (infants) CASE 2: Atrial Septal Defect (common in children) Systolic ejection murmur in pulmonic region Parasternal diastolic murmur Mild cardio w/ RAE & RVE Mild increase Pulmo bld flow

CASE 3: Patent Ductus arteriosus (common in adults) 2y/o Failure to thrive CHF Machinery like murmur in the pulmonic area inc pulmo bld flow Cardiomegaly Aorta prominent Prox asc aorta in arch of the aorta Focal aortic fil LAE & LVE

PATENT DUCTUS ARTERIOSUS

CYANOTIC HEART DISEASES **In cyanotic heart diseases, hyperaerated lungs are usually seen. 1) Normal blood flow a) Coarctation of the Aorta CASE 4 • 14 day old infant • Dyspnea • Poor feeding • Tachycardia • Peripheral cyanosis o Moderate to marked cardiomegaly o Pulmo venous congestion o Post stenotic dil. of the descending aorta 2) Increased blood flow a) Transposition of Great Arteries CASE 5 • Infants of DM • Cyanosis at birth • Acidosis • CHF • No murmur

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CXR FINDINGS: o Hyperinflated lungs o Low set HD o Narrowed based o heart o Egg shaped on side o Thymic atrophy o Cardiomegaly

b) Total Anomalous

Pulmonary Venous Return • N cardiac conf • Pulmo venous/edema • Thymic atrophy • Low set HD • Occ’l PE • Hemodiaphragm • Snowman’s sign

3) Decreased blood flow a) Tetralogy of Fallot CASE 7 • 6 mos old • Cyanosis

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Dyspnea – 1st sign

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Loud ejection murmur- L sternal angle CXR FINDINGS: o Boot shaped heart o Concave MPA o Dec hilar and central pulmo vessels o Hyperexpanded hyperlucent lungs o Thymic atrophy o Rt aortic arch

ACYANOTIC Increase PBF RVH

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Atrial Septal Defect

Ventricular Septal Defect

L to R Shunt w/ PHPN

Patent Ductus Arteriosus

PAPVR

AVSD

CYANOTIC Dec PBF

b) Tricuspid Valve Atresia Case 8 • Dyspnea • Jugular pulsations

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LVH / CVH

Loud murmur along the L sternal border Mild cardiomegaly Dec pulmo bld flow Hyperlucent lungs Concave MPA Rt aortic arch RAE and RVE

RVH

LVH

LVH / CVH

VSD w/ PS

Tricuspid Atresia

TA w/ Hypoplastic PAs

Tetralogy of Fallot

Pulmonary Atresia Hypolastic RV

Ebstein’s Anomaly

TGA w/ PS Single Ventricle w/ PS

Eisenmenger

CYANOTIC Increase PBF

c) Ebstein's Anomaly CXR findings: • Marked cardio • RAE w/ globular cardiac silhouette • Dec pulmo bld flow • Boxed heart

RVH

LVH / CVH

TAPVR

TGA w/ VSD

Hypoplastic Syndrome

Single Ventricle Truncus Arteriousus

TGA PVOD Bidirectional Shunt

CARDIAC TUMORS

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Any benign/malignant neoplasm arising primarily from the myocardium or w/in a cardiac chamber Approximately 70% are benign and 30% are malignant Metastatic tumors are not classified as cardiac tumors BENIGN MALIGNANT Myxoma Angisarcoma Lipoma Rhabdomyosarcoma Papillary Fibroadenoma Mesothelioma Hemangioma Fibrosarcoma Mesothelioma Lymphoma Fibroma Osteosarcoma

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Teratoma Granular cell tumor Neurofibroma Lympahngioma Rhabdomyoma

Thymoma Neurogenic Sarcoma Leiomyosarcoma Liposarcoma Synovial sarcoma

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LIPOMATOUS SEPTUM

INFILTRATION

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INTERATRIAL

Epicardium, the sheath of tissue that covers the heart • Myocardium, the muscles of the heart wall • Endocardium, the membrane that lines the cavities of the heart Benign Tumors Myxoma (most common) • Morphology: o Usually PEDUNCULATED w/ short, broadbased attachment o Characteristically polypoid, project into cardiac chamber and about 5+6 cm in diameter o Gelatinous/ mucoid texture and covered w/ endothelium o Arises from endocardium as small, uniform cells in myxomatous stroma o Rarely metastasizes • Location o LA 75% - most common location o RA 20% o Atrial myxomas usually arises from atrial septum o Ventricles: <10% o Multicentric: 5% o Valve: rare o Myxoma of left atrium

NOTES: Fat – bright on T1 and T2 CSF – white on T2, black on TI Malignant Tumors Sarcoma • Malignant tumor w/ wide variety of types, originating from mesenchyme • Subtype include, angisarcoma, rhabdomyosarcoma, fibrosarcoma, osteosarcoma, neutrogenic sarcoma, leiomyosarcoma, liposarcoma and synovial sarcoma • MC: RA & in mid-adulthood • Operation indicated to obtain accurate dx o Prognosis is poor, as most px have distant metastases at presentation o Adjuvant therapy may have some roles NOTES: Most common location in both Sarcoma and Lymphoma: RA

Lipoma • Well-Encapsulated Tumors, Usually Found Incidentally • Consist Of Mature Fat Cells • Most commonly occur in ATRIAL SEPTUM as part of lipomatous hyperthropy of the interatrial septum • Incidental tumors should probably be resected • PERICARDIAL LIPOMA

Papillary fibroelastomas • Would seem to be approx. equivalent to Lipoma in prevalence • Over 90% occur on cardiac valves, making them the commonest neoplasm of the valves • Many are asymptomatic, and Sx if present are usually related to embolic events • There appears to be slight predilection for leftsided valves • On MRI- it appears as a HYPO-INTENSE mobile mass

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Rhabdomyoma • Yellow-gray tumor that occur invariably in the ventricles commonly in multiple locations o Altered myocytes, often not discrete from surrounding normal myocardium • Associated w/ TUBEROUS SCLEROSIS

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METASTASES FROM AN ABDOMINAL ISLET CELL TUMOR

MC: 1o cardiac tumor in children o Cause cardiac failure from obstruction of conduction pathways and ventricular tachycardia Over 90% present before age 15, usually in the first few days of life Tumors maybe unresectable at surgery About 50% of survivors will eventually develop tuberous sclerosis

Primary Cardiac Lymphomas • Are exceedingly rare, are typically of the nonHodgkin B-cell type, and are confined to the heart or pericardium • They usually occur in immunocompromised px but are not restricted solely to this group • Presentation is w/ rapidly worsening heart failure, obstructive Sx or arrhythmias • This prognosis is invariably poor, although there have been reported remissions w/ chemotherapy • They most commonly involve the RT side of the heart, in particular the RA, w/ frequent involvement of more than one chamber and invasion of the pericardium • At MRI: they are ISOTENSE on t1-weighted images and heterogeneously HYPERINTENSE on T2-weighted images; they demonstrate heterogeneous enhancement after administration of gadolinium contrast material, w/ areas of low enhancement in the center of the lesion compared to the periphery • Secondary malignancies involving the heart are 20-40times more frequent than primary cardiac neoplasms • In autopsy studies, Px w/ known malignant neoplasms will have cardiac metastatic involvement in 10-20% of cases • Involvement in many cases may go unnoticed, but object manifestations are most commonly due to pericardial effusions and the associated impairment of the RT cardiac filling that result. • Presenting Sx may include shortness of breath, chest wall pain, and peripheral edema. As w/ other cardiac malignancies, arrhythmias may also be a feature. ***MC 1o neoplasm is BROCHOGENIC CARCINOMA followed by lymphomas, leukemia and carcinomas of the breast and esophagus ***MC site of involvement is the pericardium w/ or w/o invasion of the underlying myocardium ***In approximately 1/3 of Px w/ cardiac involvement, death will be directly attributable to the metastases as a result of pericardial tamponade, CHF, or coronary artery invasion

NON-SMALL CELL BRONCHOGENIC CARCINOMA (tumors in lungs extending to the left atrium)

DIRECT VENOUS EXTENSION OF A LEFT SIDED RENAL CELL TUMOR

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Thrombus MC mimic of cardiac neoplasm

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Most likely to be located posteriorly in the LA, commonly in the presence of atrial fibrillation, or in severely dysfunctional left ventricles. It can also be found in the right side of the heart ACUTE THROMBUS will appear bright on both T1 and T2-weighted images SUBACUTE THROMBUS will appear bright on T1-weighted images with low signal intensity areas on T2-weighted images due 2 the paramagnetic effects of methemoglobin and shortening of the T2 relaxation times. CHRONIC ORGANIZED THROMBUS will have low signal intensity on both T1 and T2weighted images due to depleted water with or without calcification of the thrombus (similar to adjacent parenchyma) Gadolinium contrast material is also useful for differentiating thrombus from tumors, as the former should not enhance.

Foreign body BULLET IN INTRAVENTRICULAR SEPTUM

REVIEW OF HEART ANATOMY

1. Atheromatous Aorta - Calcific in periphery ***EXTRACARDIAC MASSES can mimic cardiac tumors. Pericardial and bronchogenic cysts, intrathoracic neoplasms and rarely GI hernias may compress the heart to mimic a cardiac mass. Pericardial cysts

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Are congenital in origin and are usually found at the right cardiophrenic angle, although they may occur anywhere in the mediastinum. They are unilocular and contain water-based fluid w/o internal septa NOTES: Pericardial cyst – dark on TI, bright on T2 They usually demonstrate the MRI character of simple fluid and do not enhance after contrast material administration They occasionally may contain relatively proteinaqueous fluid and thus may have high signal intensity on both T1 and T2 weighted images

2. Dilated

ascending aorta

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3. Pseudoaneurysm - does not go into the area of the aorta; does not enhance

4. Aneurysm of ascending aorta - reaches > 5; emergency operation should be done

DIlated aortic arch w/ type A dissection

2) TYPE B = the tear is located just past the left subclavian artery (the blood vessel beneath the collarbone that supplies the left arm) ***Type B dissection w/ prior Type a repair:

5. Aortic dissections STANDFORD CLASSIFICATION: 1) TYPE A = the tear begins in the ascending aorta and progresses throughout the vessel, extending as far as the arteries in the leg Type A Dissection

***Dissection extends into subclavian artery

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BeBAKEY CLASSIFICATION: 1) TYPE I – involves the ascending aorta, aortic arch and descending aorta (similar to Type A of Standford) 2) TYPE II – confined to the ascending aorta (similar to Type A of Standford) 3) TYPE III – confined to the descending aorta distal to the left subclavian artery (similar to Type B of Standford) a) Type IIIa = refers to dissections that originate distal to the left subclavian artery but extend both proximally and distally, most above the diaphragm b) Type IIIb = refers to dissection that originate distal to the left subclavian artery, extend only distally and may extend below the diaphragm.

NOTES: Chest X-ray findings in pedia: • Heart is slightly elevated • Normal cardio-thoracic ratio in pedia: 0.55 (In adults: 0.5; if > than this, enlarged) • Gastric bubble just below hemidiaphragm

2 common congenital anomaly: 1. Diaphragmatic hernia • Types: Morgagni – anterior defect Bochdalek – posterior defect • A Bochdalek hernia involves an opening on the left side of the diaphragm. The stomach and intestines usually move up into the chest cavity; A Morgagni hernia involves an opening on the right side of the diaphragm. The liver and intestines usually move up into the chest cavity. • Common in the left side • Shifting of mediastinum to contralateral side • Gastric bubbles 2.

Congenital Cystic Adenomatoid Malformation (CCAM) • Multiples areas of lucencies • Gastric bubbles • Intact hemidiaphragm • No shifting of medial structures • In Ultrasound: peristalsis is seen in diaphragmatic hernia

Congenital Cystic Adenomatoid Malformation (CCAM)

PEDIATRIC NORMAL CHEST – PA VIEW NEWBORN CXR W/ DIFFUSE INFILTRATES 1. Wet lung disease Case: FT NB delivery via CS BOB during the 1st 4 hrs of life • Aka. TRANSIENT TACHYPNEA of the newborn, retention of the fetal lung fluid/ transient respiratory distress of the newborn • Mild to moderate respiratory distress sometime in the 1st 4hrs of life • Conditions that predispose the disease: a) CS b) Precipitous delivery c) Very small, hypotonic or sedated infants 3 of 11

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ROENTGENOGRAPHIC FINDINGS: a) Mild to moderate overaeration b) Symmetric parahilar patches or streaks c) Occasionally mild cardiomegaly / pleural effusions d) RT lung maybe more opacicied than the LFT e) The chest film returns to normal by 48-72 hrs of age Transient tachypnea of the newborn: Usually has non homogeneous pulmonary opacities, a normal lung volume and may have pleural fluid Transient tachypnea of the newborn/Wet Lung (TTN): Supine chest radiograph of the newborn demonstrating mild cardiomegaly and bilateral reticulonodular densities that radiate from the hila. There is atelectasis in the upper lobes.

Pulmonary Interstitial Pneumonia

Pulmonary Interstitial Pneumonia with PNEUMOMEDIASTINUM

NOTES: In utero, lungs contain amniotic fluid. Via NSD: thoracic cage is being compressed so the remaining fluid in the lungs will be squeezed out, then will be filled with air. Baby now is able to breathe (inhale). Via CS: fluid in the lungs will remain there until this would be removed

2.

Preterm baby del via NSD DOB • Small lung volume

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A finely granular (ground glass) Air bronchograms extending peripherally SURFACTANT DEFICIENCY DISEASE/HYALINE MEMBRANE DISEASE OF NEWBORN/IDOPATHIC RESPIRATORY DISTRESS SYNDROME MMC: respiratory distress in newborn infants It occurs in premature infants and also infant of DM mothers Deficiency of pulmonary surfactant superimposed on structural immaturity of the lungs Usually has a uniform distribution of pulmonary opacities, never has pleural effusions, has a decreased lung volume Complications: 1. Pulmonary interstitial pneumonia or pulmo interstitial emphysema (PIE) - interstitial infiltrates - low set hemidiaphragm - lungs are hyperaerated 2. Pneumomediastinum

History • Premature rupture of the membranes(PROM) during labor • Shortly after birth tachypnea, retratctions, and cyanosis maybe evident • Infants are often afebriel and occasionally are hypothermic • Supine chest radiograph at the 5hrs of lives demonstrate diffuse bilateral granular infiltrates 3. Neonatal Pneumonia • Group B strep pneumonia – nonhomogenous pulmonary opacities • May have pleural fluid • Lung volume is normal

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COMMON PATHOGEN: a) Stap aureus b) E.coli The infants are often afebrile & occasionally hypothermic Short after birth tachypnea, retractions and cyanosis may be evident Radiographic findings: o Symmetric pulmonary opacities o Hyperaeration are seen o Pleural effusion may be seen o Group B streptococcal pneumonia, reticulogranular densities o Consolidation occurs, it usuall is multilobular

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4. FT del via NSVD, thickly meconium • Supine chest radiograph from day one of live demonstrates bilateral, irregular coarse infiltrates

A pneumopericardium may be difficult to distinguish from a pneumomediastinum as both may have a “continuous diaphragm sign.” In a pneumopericardium, air will never dissect above the level of the main pulmonary artery, which is the superior extent

Meconium Aspiration Syndrome • Usually has nodular patchy non homogenous densities and may have pleural effusion and an increased lung volume •

Supine chest radiograph from day one of life demonstrates bilateral, irregular coarse infiltrates

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Similar to neonatal pneumonia (except there is premature rupture of membranes in neonatal pneumonia)

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Preterm chest x-ray: Ground appearance with air bronchogram

Pneumopericardium • Supine chest radiograph shows air outlining the heart, but not extending above the great vessels • Pneumopericardium, air will never dissect above the level of the main pulmonary artery, which is the superior extent of the pericardium. In a pneumomediastinum, air can dissect all the way up the mediastinim into the neck

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Trauma Pneumothorax • A pneumothorax is difficult to visualize on a supine film. The most common place to see one is in the medial basal and basilar portion of the lung. The best film to obtain to diagnose a pneumothorax is a decubitus film with the suspicious side up

Chest Infections Croup • History: 9 year old male presents with croupy cough and respiratory distress • Symmetrical subglottic narrowing on the AP view • Pencil-shaped configuration of the subglottic edema • “Steeple sign” • An acute infection of the lower air passages • Children below age of 3s exceptions • Parainfluenza – most common pathogen

Pneumomediastinum (PMS)

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Epiglottitis • History: A 2 year old boy is brought to the emergency department at 2:30 am • Complaining of throat pain, not drinking, drooling, difficulty swallowing, coughing, naseal congestion, and fever • The throat pain, difficulty drinking, and drooling started in the afternoon and have worsened over the night. He has had the coughing and nasal congestion for 3 days now • The black arrow points to the tip of the epiglottis and the white arrow points to the preepiglottic space (vallecula)

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The epiglottis should normally be thin or triangular in appearance. In this view, it appears to be rounded and somewhat thumb-like, which is similar to the classic radiographic description of epiglottitis

Tuberculosis • History: 4 year old female exposed to active tuberculosis by a sibling

Atelectasis or 20 to Hilar lymphadenopathies • Manifests with fever • Chest x-ray: segmented opacities in right middle lobe

Cystic Fibrosis • Hx: 11 y/o male being evaluated for a surgical procedure • Chest x-ray: hyperlucent with multiple infiltrates Nodules in hilum

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Upper, middle, and lower lungs show thickened interlobular septa and brochiectasis and perhaps some hilar lymphadenopathy TB

Empyema • Hx: 7 year old male with fever and nonproductive cough

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Right upper lobe consolidation and a large right pleural effusion Chest x-ray: complete opacified right lung

An enhanced chest CT exam shows a large right pleural effusion with an enhancing rim with associated consolidation and atelectasis of the lung Enhancement of pleura

Pulmonary Abscess

Pulmonary abscess • Hx: an 8 year old male with fever, cough, and bad breath • Multi-cavitary lesions

Pleural Effusion (Lateral Decubitus)

*show a cavitary lesion with an air fluid level in the right lower lobe

Subpulmonic Effusion - hemidiaphragm elevated when comparted to left 7 x 7.5 x 8 cm intrapulmonary fluid collection in the right lower lobe with thick nodular walls and air fluid level

Round Pneumonia

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Lymphoma - Bilateral nodular opacities, smooth border, mediastinal pathology

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