Cardiovascular System

Cardiovascular System

History Children do not present with typical features of congestive heart failure that are seen in adults Infants present with feeding difficulties, easy fatigability, sweating while feeding, and rapid respirations Older child may have SOB and dyspnea on exertion Orthopnea, edema, and nocturnal dyspnea are uncommon

Physical examination Heart rates according to the ages Height and weight assessed to determine proper growth Upper and lower extremity blood pressures Palpation of upper and lower extremity pulses Cyanosis and clubbing from hypoxia Hepatomegaly suggestive for right sided heart failure Prominent precordium with cardiomegaly Palpation may show cardiac impulses and thrills Murmur (graded I to VI) Rales: may indicate pulmonary edema and left sided heart failure

Features of cardiac exam Apical heave Substernal thrust Hyperdynamic precordium Silent precordium Thrill Ejection click S3 Gallop

Systolic ejection murmur Pansystolic murmur Continuous murmur To-and-fro murmur Late systolic murmur Diastolic murmur Venous hum Wide pulse pressure (>40 mm Hg) Narrow pulse pressure Narrow split S2

Location of murmurs Upper right sternal border: AS Upper left sternal border: PS, pulmonary ejection murmur ASD PDA Coarctation of aorta TAPVR

Lower left sternum border: VSD Still murmur TOF Tricuspid atresia Apex: Still murmur MVP Idiopathic, hypertrophic subaortic stenosis (IHSS), asymmetric septal hypertrophy

Diagnostic tests: CXR ECG Echocardiography MRI Cardiac catheterization Angiography Exercise testing Cardiac embryology: Helpful to understand congenital cardiac lesions

Fetal circulation

Fetal circulatio n

Also known as functional, normal, insignificant, or flow murmurs These result from flow through a normal heart, vessels, and valves Most innocent murmurs heard between 3 and 7 years of age More than 30% of children may have an innocent murmur heard at some time in their lives

Presentation Innocent murmurs usually heard on routine physical examination Also easily heard during times of increased cardiac output such as during fever, infection, or anxiety Never diastolic A soft, vibratory or musical systolic ejection murmur best heard at the left lower to midsternal border Never greater than grade 2/6 Diagnostic tests: further evaluation not needed

Treatment No treatment necessary; reassurance of parents and patient D/D Still murmur: •The most common innocent murmur of early childhood; heard between 2 and 7 years of age •Musical or vibratory, short, high-pitched, grade I–III early systolic murmur •Loudest midway between the apex and the lower left sternal border

Pulmonary flow murmurs: •The most common innocent murmur in older children; heard from age 3 years onward •High pitched, blowing, early systolic murmurs best heard in the second left parasternal space with the patient lying down Venous hum: •Heard in the neck or anterior chest •Heard in systole and diastole but can disappear with compression of the jugular vein

Occurs in about .5 - 0.8/100 live births Most lesions occur between 15 and 80 days of gestation Diagnosis usually made early in life: 50 – 60% of patients diagnosed by 1 month of age Congenital heart disease can be classified as:

Acyanotic heart disease: Ventricular septal defect (VSD) Atrial septal difect (ASD) Patent ductus arteriosus (PDA) Endocardial cushion defects (A-V canal) Coarctation of aorta Cyanotic heart disease: Tetralogy of Fallot (TOF) Transposition of the great vessels Pulmonary atresia Tricuspid atresia Total anomalous pulmonary venous return (TAPVR) Truncus arteriosus (TA)

The most common congenital cardiac malformation Accounts for 25% of congenital heart disease Presentation Depends on the size of the defect, and the pulmonary flow and pressure Small defects limit the size of the shunting Large defects allow for more left to right shunting, which is determined by differences in systemic and pulmonary vascular resistances

Persistently high pulmonary blood flow leads to increased pulmonary vascular resistance secondary to medial hypertrophy of the pulmonary vessels When the pulmonary vascular resistance equals the systemic vascular resistance, the previously left to right shunt becomes bidirectional or right to left, leading to cyanosis, a condition known as Eisenmenger physiology Small defects are usually asymptomatic VSD murmur is typically a harsh holosystolic murmur at the left lower sternal border Thrill is frequently associated Large defects can cause dyspnea, feeding, difficulties, poor growth and sweating

Diagnostic tests CXR: reveals a large heart, but normal in small defects ECG: LVH; biventricular hypertrophy and notched P waves in large defects Echo: reveals the defect and estimates the shunt size Treatment Small defects close spontaneously in 30 – 50% of cases by 2 years of age and require no therapy Antibiotic prophylaxis for endocarditis required Medical management includes controlling heart failure, promoting normal growth, and preventing pulmonary vascular disease

Surgical closure recommended for failure of medical management, or large defects with pulmonary hypertension that has not yet become severe Small VSDs and surgical repair without residuals have an excellent long-term prognosis Complications Large defects can result in heart failure and failure to thrive Endocarditis Pulmonary hypertension

ASD occurs anywhere along the atrial septum Ostium secundum defects are the most common Presentation Many patients are asymptomatic Exercise intolerance may develop in older children A wide fixed split of S2 Systolic ejection murmur heard in the left mid and upper sternal border Usually there is no thrill

Diagnostic tests CXR: shows an enlarged right atrium and ventricle, depending on the sixe of the shunt EC G: Right atrial enlargement and right ventricular conduction delay (rSR’) Echo: right ventricular volume overload and demonstrates ASD Treatment: Surgical closure with open heart surgery or occlusion devices Complications Symptoms do not appear until the third decade Endocarditis is rare and prophylaxis not recommended Atrial dysrhythmias, valvular insufficiency (mitral, tricuspid), and heart failure may occur

PDA results from failure of closure of the ducts arteriosus Leads to blood flow from the aorta to the pulmonary artery PDA more common in girls (2:1) Associated with maternal rubella infection Common in premature infants PDA can beneficial in providing pulmonary blood flow when there is an associated right ventricular outflow tract obstruction, or in supplying systemic flow in coarctation of the aorta

Presentation Small PDA asymptomatic Large PDA can cause heart failure similar to large VSD Wide pulse pressure and bounding arterial pulses Apical heave Thrill at the second left intercostal space Machinery or to-and-fro murmur heard in both systole and diastole Can be less prominent or not heard in diastole in infants or in the presence of increased pulmonary vascular resistance

Diagnostic tests CXR: shows a prominent pulmonary artery and increased pulmonary vascular markings Heart size may be normal or slightly enlarged Echo: shows PDA Treatment: PDA in premature infants may close sponateuously or respond well to indomethacin They rarely close spontaneously after infancy, and surgical or catheter closure recommended Complications CHF and IE

•A constriction of aorta arising at any point •Majority (98%) occur just below the origin of the left subclavian artery •Common in boys (2:1) •An increased incidence in Turner syndrome, which is associated with bicuspid aortic valve

Presentation •A coarctation may be missed in a newborn while the ductus is open •Severe coarctation in infancy presents with congestive heart failure, metabolic acidosis, and lower body hypoperfusion •Upper or lower extremity pulse delay a classic sign •Hypertension in upper extremities and hypotension in the lower extremities •Hypertension in the right upper extremity versus the left upper extremity suggests a coarctation around the left subclavian •A short systolic murmur heard along the left sternal border at the third to fourth intercostal space

Diagnostic tests CXR: Severe coarctation in infancy reveals cardiac enlargement and ↑ pulmonary vascular markings Older patients show rib notching from enlargement of the intercostals providing collateral blood flow ECG: In infancy, RVH In later childhood, LVH Echo: Narrowed segment and a hypopulsatile descending aorta

Treatment Surgical correction as soon as the patient si stable In neonates, ductus is kept open with an infusion of prostaglandin E1 Complications Hypertension, which can lead to premature CAD, heart failure, encephalopathy, and intracranial hemorrhage Adults at risk for endocarditis

Tetralogy of Fallot (TOF) 1)Pulmonary stenosis 2)Right ventricular hypertrophy 3)VSD 4)Overriding of aorta

Presentation •Symptoms depend on the size of VSD and degree of RVOT obstruction •Acyanotic (pink) tetralogy occurs when there is sufficient pulmonary blood flow caused by mild obstruction and the shunting across the VSD is balanced •Typically, patients present with cyanosis, delayed growth and development and dyspnea •Paroxysmal hypercyanotic attacks (hypoxic blue spells) •Clubbing in chronic hypoxia •S2 single or very soft (because of the pulmonary stenosis) •Loud, harsh systolic ejection murmur

Diagnostic tests CXR: A boot-shaped heart (couer en sabot) with up tilted apex Lung fields clear reflecting decreased pulmonary blood flow ECG: RVH and right-axis deviation Echo: Anatomic abnormalities

Treatment Maintaining the ductus open in severe right-sided obstructive lesions, but surgical correction is the definitive treatment Blue spells treated with knee chest position, sedation, O2 and avoiding acidosis B-blockade with propranolol, appropriate fluid status, and maintaining the hematocrit at 55-65% helpful Complications Cerebral thrombosis with extreme polycythemia and dehydration Patients usually less than 2 years of age Brain abscess common in patients older than 2 years Tet patients at higher risk for bacterial endocarditis

An inflammation of the myocardium Risk factors/ etiology •Infetctious causes Viral (Adneovirus, Coxsackievirus B) Bacteria (Diphtheria) Rickettsia Fungi Parasites •Connective tissue disease •Granulomatous disease •Toxins

Presentation •Heart failure •Arrhythmia •Sudden death •Infants have more acute and fulminant presentation •Usually preceded by a viral infection •Patient can present with fever, heart failure, respiratory distress and cyanosis

Diagnostic tests •ESR, creatine kinase, LDH may all be elevated •Serum viral titers helpful only if positive •PCR to identify specific viruses •CXR shows a large heart and pulmonary edema •ECG shows sinus tachycardia, reduced ZRS complex, and abnormal S and ST waves •Echocardiograhy show poor ventricular function and possible pericardial effusions and absence of congenital heart disease and of coronary involvement •Endomyocardial biopsy confirms the diagnosis of myocarditis

Treatment •Management of heart failure and arrhythmias •Pericardiocentesis if tamponade •Role of steroid controversial •Refractory heart failure ultimately treated with heart transplantation Prognosis •Most patients do very poorly without therapy •Spontaneous resolution can occur

Caused after pharyngeal infection with group A β hemolytic streptococci (GABHS) Most commonly seen in those who are susceptible to infections by GABHS, namely, 5 to 15 yeas old Acute glomerulonephritis (AGN) can follow streptococcal infections of either the pharynx or the skin—in contrast to rheumatic fever, which follows pharyngeal infection only.

Presentation Usually presents 1-3 (2-6 weeks) weeks after a preceding streptococcal pharyngitis Jones criteria for diagnosis Carey coombs murmur A short diastolic rumbling murmur heard in mitral valvulitis associated with acute rheumatic fever

Jones Criteria (Modified) for Diagnosis of Rheumatic Fever Major manifestations    Carditis   Polyarthritis (migratory)   Sydenham chorea   Erythema marginatum   Subcutaneous nodules Minor manifestations    Clinical      Previous rheumatic fever or rheumatic heart disease     Polyarthralgia     Fever   Laboratory      Acute phase reaction: elevated erythrocyte sedimentation rate, Creactive protein, leukocytosis     Prolonged PR interval Plus  Supporting evidence of preceding streptococcal infection, that is, increased titers of antistreptolysin O or other streptococcal antibodies, positive throat culture for group A Streptococcus 

Diagnosis •Two major criteria or one major and two minor criteria plus preceding streptococci infection, •Chorea without any other explanation If arthritis one of the presenting major criteria, then arthralgia cannot be one of the presenting minor criteria

Treatment •Treatment of the streptococcal infection penicillin V (phenoxymethyl penicillin) is the drug of choice. For children weighing less than 27 kg, the regimen is 250 mg, given orally two or three times a day for 10 days a single dose of benzathine penicillin G, given IM (0.6 million units for children weighing < 60 lb [27.2 kg] and 1.2 million units for children weighing > 60 lb [27.2 kg]) For penicillin-allergic patients : erythromycin estolate for 10 days; azithromycin once daily for 5 days

Treatment (contd…) •Salicylates to control the arthritis and to treat carditis without failure •Steroids used when there is carditis with heart failure •Heart failure treated conventionally Complications Valvular disease In order of frequency, mitral, aortic, tricuspid, and pulmonary valves

Prophylaxis of Streptococcal infection: Benzathine penicillin G, 1.2 million units IM every 3-4 weeks Oral prophylaxis: sulfadiazine, 0.5 g (if < 27 kg) or 1 g (if > 27 kg)/d; penicillin V, 200,000 units twice daily; or erythromycin, 250 mg twice daily Prophylaxis is continued for at least 5 years or until age 21 years (whichever is longer) if carditis is absent

Risk factors/ etiology •Streptococcus viridans the most common cause •Staphylococcus aureus common, if no underlying heart disease •S. Viridans more common after dental procedures •Pseudomonas aeruginosa and Serratia marcescens more commonly in intravenous drug abusers •Fungal causes after open-heart surgery Endocarditis most often associated with congenital or rheumatic heart disease Surgical or dental procedures often the predisposing incident The highest risk occurs in patients with high-velocity blood flow, ie, VSD and left-sided obstructive lesions

Presentation •Onset of symptoms may be acute or insidious •Fever, chills, arthralgias, myalgias, development of new murmurs, splenomegaly, and petechiae •Neurologic complications associated with S. aureus •Skin manifestations occur later in the disease and probably represent a vasculitis Osler nodes (tender nodules on the finger and toe pads) Janeway lesions (painless hemorrhagic lesions on palms and soles) Splinter hemorrhages (linear lesions beneath the nails)

Diagnosis •Positive blood culture •Leukocytosis, increased ESR, anemia •Echocardiography may reveal vegetations on the valves Treatment •Antibiotics for 4 – 6 weeks •Treat Congestive heart failure, if present •Prevention with antibiotic prophylaxis before and after dental or surgical procedures Complications •Mortality 20 – 25% •Heart failure, systemic embolisms

Prophylaxis and cardiac conditions

Prophylaxis and cardiac conditions

Procedures recommended for Prophylaxis

Antibiotics used for prophylaxis

Systemic hypertension defined as blood pressure above the 95th percentile for age on repeated measurements over a 6-week period Risk factors/ etiology Primary hypertension •No known underlying cause •Predisposing factors include heredity, salt intake, stress, and obesity •More commonly seen in adolescent and adults

Secondary hypertension •Caused by an associated disease •More common in infants and younger children •Common cause in children is Renal disease (75 – 80%) A prior UTI seen in 25 – 50% of cases, often related to an obstructive lesion of the urinary tract In newborns, a H/O umbilical artery catheterization may be elicited, with resultant thrombus of the renal artery

Presentation •Usually no symptoms, especially in adolescents with essential hypertension, •Diagnosed on routine examination •Headaches, dizziness, vision changes, and seizures may be present Diagnostic tests •Blood pressure should be measured over several visits and compared with normal values for age •BP should be taken in all extremities to rule out coarctation of aorta

•All children with secondary hypertension should have a renal evaluation, including culture, ultrasound, renin levels, BUN, and creatinine •Echo assesses ventricular function and size Treatment •Same as in adults: diet, exercise, and medications Pharmacologic management includes: Diuretics, β -blockers, ACE inhibitors, Calcium channel blockers