I. Definition Thirty 30 mm Hg has been used as the normal upper limit for systolic PA pressure and 25 mm Hg as the normal upper limit for mean PA pressure when measured directly by cardiac catheterization in adults and children older than 3 months of age at sea level. Thus a diagnosis of pulmonary hypertension (PH) was made when mean PA pressure was ≥25 mm…
I. Definition For adults, the 2017 Guidelines by the American College of Cardiology, American Heart Association, and several other organizations jointly revised the earlier definition and classification of hypertension (HTN) of 2003. The following are the new classification (Whelton et al., 2018). Table 23.1 shows different levels of blood pressure (BP) readings by the new definition of elevated BP. 1. Normal BP is defined as <120…
I. Definition Palpitation is one of the most common cardiac symptoms encountered in medical practice but it poorly corresponds to demonstrable abnormalities. The term palpitation is used loosely to describe an unpleasant subjective awareness of one’s own heartbeats. This usually occurs as a sensation in the chest of rapid, irregular, or unusually strong heartbeats. II. Causes Many palpitations are often not serious, but they may indicate…
I. Definition The brain depends on a constant supply of oxygen and glucose for normal function. Significant alterations in the supply of oxygen and glucose to the brain may result in a transient loss of consciousness. 1. Syncope is a transient loss of consciousness and muscle tone with a fall. 2. Presyncope is the feeling that one is about to pass out but remains conscious with…
Although chest pain does not indicate serious disease of the heart or other systems in most pediatric patients, in a society with a high prevalence of atherosclerotic heart disease, it can be alarming to the child and parents. Physicians should be aware of the differential diagnosis of chest pain in children and should make every effort to find a specific cause before making a referral to…
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A pacemaker is a device that delivers battery-supplied electrical stimuli over leads to electrodes that are in contact with the heart. It primarily treats bradycardia. An implantable cardioverter defibrillator (ICD) is a multiprogrammable antiarrhythmic device for treating ventricular tachycardia and ventricular fibrillation. ICDs also possess pacemaking capability to treat bradycardia. I. Pacemakers in Children For pacemakers, the electrical leads are inserted either directly over the epicardium…
AV block is a disturbance in conduction between the normal sinus impulse and the eventual ventricular response. The block is assigned to one of three classes, according to the severity of the conduction disturbance. 1. First-degree AV block is a simple prolongation of the PR interval but all P waves are conducted to the ventricle. 2. In second-degree AV block, some atrial impulses are not conducted…
Normal heart rate varies with age: the younger the child, the faster the heart rate. Therefore the definitions used for adults of bradycardia (fewer than 60 beats/min) and tachycardia (above 100 beats/min) have little significance for children. A child has tachycardia when the heart rate is beyond the upper limit of normal for age and bradycardia when the heart rate is slower than the lower limit…
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Many systemic diseases may have important cardiovascular (CV) manifestations. The CV manifestations usually are evident when the diagnosis of the primary disease is made, but occasionally CV manifestations may precede evidence of the basic disease. In this chapter, CV manifestations of selected systemic diseases are presented. I 22q11 Deletion Syndrome (D i george Syndrome) A Description This syndrome, formerly known as DiGeorge syndrome, occurs in both…
I. Prevalence 1. Cardiac tumors in the pediatric age group are very rare. A large portion of pediatric primary heart tumors (about 70%) are seen in patients younger than 1 year. 2. Relative frequency of cardiac tumors in the pediatric age group is shown in Table 14.1 . a. In infants younger than 1 year, more than 50% are rhabdomyomas, followed by fibromas (25%). b. In…
Valvular heart disease is either congenital or acquired. Many congenital valvular abnormalities are associated with other major defects. A relatively isolated form of valvular heart disease is rheumatic in origin, which still occurs in some parts of the world. Among rheumatic heart disease, mitral valve involvement occurs in about three-fourths and aortic valve involvement in about one-fourth of cases. Rheumatic involvement of the tricuspid and pulmonary…
I. Infective Endocarditis (Subacute Bacterial Endocarditis) A. Prevalence Subacute bacterial endocarditis (SBE) affects 0.5:1000 to 1:1000 hospital patients, excluding those with postoperative endocarditis. B. Pathology and Pathogenesis 1. Two factors are important in the pathogenesis of IE: (a) structural abnormalities of the heart or great arteries with a significant pressure gradient or turbulence, with resulting endothelial damage and platelet-fibrin thrombus formation; and (b) bacteremia, even if…
Primary myocardial disease affects the heart muscle itself and is not associated with congenital, valvular, or coronary heart disease or systemic disorders. Cardiomyopathy has been classified into three types based on anatomic and functional features: (1) hypertrophic, (2) dilated (or congestive), and (3) restrictive ( Fig. 11.1 ). In 1995, two other categories were added: arrhythmogenic cardiomyopathy and left ventricular noncompaction. Different subtypes of cardiomyopathy are…
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I. Anomalous Origin of the Left Coronary Artery (Bland-White-Garland Syndrome, Alcapa Syndrome) A. Pathology and Pathophysiology The left coronary artery (LCA) arises abnormally from the main PA. Postnatal decrease in the PA pressure results in ineffective perfusion of the LCA, producing ischemia and infarction of the LV that is normally perfused by the LCA. B. Clinical Manifestations 1. The newborn patient is usually asymptomatic until the…
A. CYANOSIS I. Pathophysiology of Cyanosis Before discussing individual cyanotic CHD, a brief review of pathophysiology of cyanosis is in order. II. Causes of Cyanosis Cyanosis is a bluish discoloration of the skin and mucous membranes resulting from an increased concentration of reduced hemoglobin to about 5 g/100 mL in the cutaneous veins. This level of reduced hemoglobin in the cutaneous vein may result from either desaturation of…
I. Pulmonary Stenosis A. Prevalence Isolated PS occurs in 4% to 8% of all CHDs. B. Pathology and Pathophysiology 1. PS may be valvular (90%), subvalvular (infundibular), or supravalvular (i.e., stenosis of the main PA). Stenosis of the PA branches is presented in Chapter 10 . a. In valvular PS, the pulmonary valve is thickened, with fused or absent commissures and a small orifice. A poststenotic…