Parents should be alarmed by unsatisfactory ponderal gain, cyanosis (permanent or due to the effort of feeding), profuse perspiration, apathy, fast or difficult breathing, persistent wheezing or coughing in the supine position. Infants may sleep more than normal and have diminished activity in general [1].

A particular problem is represented by tet spells. These appear in children with cyanotic CHD such as tetralogy of Fallot, severe pulmonary stenosis or atresia or other decreased pulmonary blood flow malformations. Tet spells consist of inconsolable crying with increasing cyanosis and hyperpnoea and may lead to the loss of consciousness and even death. Older children instinctively adopt a squatting position, thus increasing systemic vascular resistance and blood flow to the lungs [2].

Blood saturation may differ in different regions of the body, so upper extremities may be more pink than lower extremities (differential cyanosis, found in patent ductus arteriosus with pulmonary hypertension or preductal coarctation of the aorta associated with patent ductus arteriosus ) or more bluish than the lower extremities (reversed differential cyanosis, found in transposition of the great arteries with patent ductus arteriosus and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation [3].

Presentation of children with ductal-dependent CHD dramatically changes as the ductus arteriosus changes. Thus, symptoms depend on whether the pulmonary circulation or the systemic circulation depend on the patency of the ductus arteriosus.

• Pediatric Disease

  Written with more than 425 chapters, the book will encompass virtually all pediatric subspecialties, covering every pediatric disease and organ system. [books.google.com]

• Inflammation

  Theoretically, whole blood should improve haemostasis and decrease systemic inflammation in comparison with packed red cells. [cochranelibrary.com]

• Tachypnea

  […] ventilation with mean airway pressure > 6–8 cm H2O < 30% Use of any supplemental O2 Use of any CPAP or mechanical ventilation Significant abnormalities of heart rate or respiratory rate† < 20% Low reticulocyte count and symptoms of anemia (eg, tachycardia, tachypnea [merckmanuals.com]

  Management 4 See also 5 References 6 External links Signs and symptoms [ edit ] Clubbing The patient assuming a crouching position Cyanosis - bluish face, particularly the lips; and bluish fingers and toes Crying Crabbiness/irritability Tachycardia Tachypnea [en.wikipedia.org]

  In the absence of a VSD, as the PDA closes, patients may develop intense cyanosis, tachypnea and tachycardia. [hawaii.edu]

• Heart Disease

  There are little data to attest their accuracy in cyanotic congenital heart disease. Aims We aimed to assess the accuracy of a commonly used probe in children with cyanotic congenital heart disease. [ncbi.nlm.nih.gov]

• Cyanosis

  Recent evidence suggests that deranged endothelial function, a sequel of chronic cyanosis, could be an important factor in the pathogenesis of cyanosis-associated cardiovascular risk. [ncbi.nlm.nih.gov]

  Tet spells consist of inconsolable crying with increasing cyanosis and hyperpnoea and may lead to the loss of consciousness and even death. [symptoma.com]

  Important flags on history include: Cyanosis Timing and location (peripheral or central) of cyanosis, clubbing Refractory cyanosis if fails to improve with oxygen therapy Fainting or cyanotic spells Cyanosis occurring with exertion, emotions, and/or bearing [learn.pediatrics.ubc.ca]

  Signs & Symptoms Onset Cyanosis (mild) Cyanosis (mod to severe) Cyanosis (severe) + Resp distress with Low CO Cyanosis (mild to mod) with CHF Immediately after birth Initiation of pulmonary circulation Truncus arteriosus DORV without PS DILV without PS [ommegaonline.org]

• Heart Murmur

  See also separate Heart Murmurs in Children article: Not all murmurs are pathological. Murmurs heard in the first week to months of life are more likely to be due to congenital heart disease than those discovered in later childhood years. [patient.info]

  Auscultation findings may include tachycardic or bradycardic sounds;heart murmurs- their location, intensity, musical qualities and lack of variability with respiratory movements may suggest the underlying defect; paradoxically or fixed split, single [symptoma.com]

  Signs and symptoms [ edit ] Shortness of breath Congested cough Diaphoresis Fatigue Frequent respiratory infections Machine-like heart murmur Tachycardia Tachypnea Respiratory distress Mild cyanosis (in right sided heart failure) Poor growth and development [en.wikipedia.org]

  The doctor will listen to the heart and lungs with a stethoscope. Abnormal heart sounds, a heart murmur, and lung crackles may be heard. [nlm.nih.gov]

  On the other hand, they do not have to be accompanied by murmur. Murmur is usually wrongly considered as a leading sign of heart disease ( 16 ). [ncbi.nlm.nih.gov]

• Systolic Murmur

  There may be a systolic murmur at the left sternal border or an apical aortic ejection click. A diastolic murmur of truncal insufficiency may be heard along the left sternal border. [hawaii.edu]

  Mod-severe: mmHg, > 60mmHg Prominent jugular a-wave RV lift, RV heave Split 2nd hrt sound Ejection click, followed by systolic murmur. [slideplayer.com]

• Ejection Murmur

  Mod-severe: mmHg, > 60mmHg Prominent jugular a-wave RV lift, RV heave Split 2nd hrt sound Ejection click, followed by systolic murmur. [slideplayer.com]

  There may be a systolic murmur at the left sternal border or an apical aortic ejection click. A diastolic murmur of truncal insufficiency may be heard along the left sternal border. [hawaii.edu]

• Dizziness

  We report the case of a cyanotic congenital heart disease patient with vertigo and dizziness who underwent a modified B-T fistula on childhood and developed, many years later, a vertiginous syndrome probably of vascular origin. [ncbi.nlm.nih.gov]

  […] or uncontrolled diabetes in pregnant women and alcohol consumption during pregnancy Cyanotic Heart Diseases signs and symptoms may include shortness of breath, very low levels of oxygen ‘spells’, loss of weight, puffy face and eyes, clubbed fingers, dizziness [dovemed.com]

  Call your provider if your baby has: Bluish skin (cyanosis) or grayish skin Breathing difficulty Chest pain or other pain Dizziness, fainting, or heart palpitations Feeding problems or reduced appetite Fever, nausea, or vomiting Puffy eyes or face Tiredness [nlm.nih.gov]

• Hyperactivity

  Inspection and palpation may reveal a hyperactive precordium, dextrocardia, heart enlargement or thrill. [symptoma.com]

  Pulse s Aortic Coarctation (Interrupted aortic arch) Asymmetric Pulse s Aortic Stenosis Decreased pulses Left Ventricular Hypertrophy Hypoplastic Left Heart Syndrome Most common CHF cause in first week Decreased pulses Right Ventricular Hypertrophy Hyperactive [fpnotebook.com]

  • Cyanosis does not occur unless Pulm HTN or R-to-L shunt is present. 35 Atrial Septal Defect Physical finding RV heave (RV systolic lift • mild left precordial bulge (hyperactive precordium); RV heave (RV systolic lift is palpable at the LSB); Feel [slideplayer.com]

The clinician should take a short history about the presence of CHD or sudden death in other family members. Inspection and palpation may reveal a hyperactive precordium, dextrocardia, heart enlargement or thrill. The heart impulse might be displaced (if the right ventricle is overloaded) or prominent (when the left ventricle has a pressure or volume overload). Auscultation findings may include tachycardic or bradycardic sounds;
heart murmurs- their location, intensity, musical qualities and lack of variability with respiratory movements may suggest the underlying defect; paradoxically or fixed split, single or inaudible sounds: S2 may be single in conditions associated with pulmonary hypertension or great vessels valve abnormalities: aortic or pulmonary atresia or severe stenosis or truncus arteriosus; abnormal supplementary heart sounds: early systolic (aortic or pulmonary stenosis) or mid systolic clicks (mitral valve prolapse, Ebstein’s anomaly), atrial or ventricular gallop may also occur. Pericardial friction rubs suggest pericarditis, while murmurs may be absent in the presence of severe ventricular dysfunction or pulmonary hypertension [4] [5] [6].
Liver enlargement should also be evaluated, keeping in mind that the inferior edge is normally felt 1-3 cm below the rib cage in newborns. The symmetry and amplitude of pulses should be routinely observed, in order not to miss associated abnormalities such as coarctation of the aorta or interrupted aortic arch.

Numerous professional cardiology associations recommend routine pulse oximetry newborn screening. The sensor should be placed on the right hand (to measure preductal saturation) or the foot (to measure postductal saturation) [7] [8] [9] [10] [11] [12].

An electrocardiogram may be normal or may show arrhythmia or congenital heart block, bundle branch blocks or cavity enlargement.

Thoracic radiography should be used to evaluate the location of the heart, vascular pulmonary markings, and cardiac silhouette: "boot" shape in tetralogy of Fallot, "egg-on-string" in transposition of great arteries, or "snowman" in total anomalous pulmonary venous return.

Echocardiography is performed in patients with signs of cardiogenic shock or abnormal findings on the ECG or thoracic radiography. Echocardiography is able to accurately measure cavities and walls, pressure gradients and velocities, and describe defects or exclude CHD as the cause of cyanosis.

If doubt arises from echocardiographic studies or if symptoms do not match echocardiographic data, cardiac computer tomography and magnetic resonance might be useful. However, these are not available in all centers, especially in underdeveloped countries. In this situation and in cases where it is necessary to measure pulmonary vascular resistance to determine if surgery is appropriate, cardiac catheterization is indicated. However, given that this method involves radiation exposure, it should only be used in cases where cardiac anatomy or function is not properly defined by less invasive methods, such as low flow lesions that make pulmonary resistance calculation difficult [13].

• Hematocrit Increased

  While polycythemia is helpful, when hematocrit exceeds 70%, it becomes counter-productive because it produces hypervolemia, causes increased yield shear stress and high blood viscosity which in turn increases peripheral vascular resistance, reduces cardiac [omicsonline.org]

OBJECTIVE: This study was planned to determine the effects of iron treatment in children with cyanotic congenital heart disease. [ncbi.nlm.nih.gov]

[…] hypertension * prevention and treatment of infective endocarditis * treatment of pericardial disease * and rehabilitation of the patient with cardiovascular disease. [books.google.com]

When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. [ncbi.nlm.nih.gov]

Prognosis is good after surgery. Prognosis is poor in neonates with obstructive TAPVR. Long term follow up is needed to assess restenosis and late arrhythmias. [hawaii.edu]

There appears to be an etiologic connection between HOA and CCHD. [ncbi.nlm.nih.gov]

Causes: Most Common Etiologies Cyanotic Congenital Heart Disease (5 T's, with 1-5 mnemonic) Truncus Arteriosus (1 vessel) Transposition of the Great Vessels (2 vessels switched) Tricuspid valve atresia (3 valve cusps) Tetralogy of Fallot (4 components [fpnotebook.com]

When faced with a blue baby in the ED, there are 4 etiologies to always consider. [emergencymedicinecases.com]

OBJECTIVE: To compare the risk of respiratory syncytial virus (RSV)-associated hospitalization and analyze the epidemiology of RSV infection in patients with cyanotic and acyanotic congenital heart disease (CHD), we analyzed the nationwide health insurance [ncbi.nlm.nih.gov]

Epidemiology The reported total birth prevalence of congenital heart disease in Europe is 8.2 per thousand live births which is higher than reported birth prevalence in the USA (6.9 per thousand live births). [patient.info]

The epidemiology and genetics of congenital heart disease. (2001) Clin Perinatol 28(1): 1-10. [ommegaonline.org]

There are more than 1.8 million adults living with congenital heart defects. [40] Epidemiology [ edit ] Congenital heart anomalies deaths per million persons in 2012 0-8 9-12 13-23 24-31 32-39 40-47 48-50 51-56 57-63 64-124 Heart defects are among the [en.wikipedia.org]

Epidemiological studies have confirmed this decreasing mortality and prolonged survival in young patients resulting in a growing and ageing population with congenital heart disease (Khairy 2010; Knowles 2012; Wren 2001). [cochranelibrary.com]

In this article, we discuss the physiological and mechanical consequences of compensatory erythrocytosis and possible pathophysiological mechanisms of vascular dysfunction in chronic cyanosis. [ncbi.nlm.nih.gov]

Reorganized to mirror your changing practice, each cardiovascular-problem-based section offers a chapter on pharmacologic treatment (with a minimum of pathophysiology)...a chapter on catheter-based or minimally invasive interventions...and a discussion [books.google.com]

In the following paragraphs, the pathophysiology of each category is discussed. [ommegaonline.org]

Keeps you current with brand-new chapters on gene therapy and stem cell therapy * unstable and chronic stable angina * management of unstable angina and non-ST-elevation myocardial infarction * devices for heart failure and arrhythmias * prevention of [books.google.com]

Early intervention in patients with CCHD is desirable in order to prevent development of complications which worsen the prognosis. [ncbi.nlm.nih.gov]

Can congenital heart defects be prevented? Most congenital heart defects cannot be prevented. A woman can do some things to reduce the risk of congenital heart defects though. [americanpregnancy.org]

Prevention Although many forms of congenital heart disease are not currently preventable, the avoidance of known risk factors such as drugs and alcohol during pregnancy will help to reduce the risk. [patient.info]

Surgical intervention, to prevent LV dysfunction. Angioplasty is used by some centers. [slideplayer.com]

1. Aisenberg RB, Rosenthal A, Nadas AS, Wolff PH. Developmental delay in infants with congenital heart disease. Correlation with hypoxemia and congestive heart failure. Pediatr Cardiol 1982; 3:133.
2. Park MK. Pediatric cardiology for Practitioners.4th end. Mosey, St. Louis. 2004; 123
3. Yap SH, Anania N, Alboliras ET, Lilien LD. Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous pulmonary venous connection. Pediatr Cardiol. 2009 Apr;30(3):359-62
4. Rein AJ, Omokhodion SI, Nir A. Significance of a cardiac murmur as the sole clinical sign in the newborn. Clin Pediatr (Phila). 2000; 39:511.
5. Hansen LK, Birkebaek NH, Oxhøj H. Initial evaluation of children with heart murmurs by the non-specialized paediatrician. Eur J Pediatr. 1995; 154:15.
6. McCrindle BW, Shaffer KM, Kan JS, et al. Cardinal clinical signs in the differentiation of heart murmurs in children. Arch Pediatr Adolesc Med. 1996; 150:169.
7. de-Wahl Granelli A, Wennergren M, Sandberg K, et al. Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ. 2009; 338:a3037.
8. Meberg A, Brügmann-Pieper S, Due R Jr, et al. First day of life pulse oximetry screening to detect congenital heart defects. J Pediatr. 2008; 152:761.
9. Riede FT, Wörner C, Dähnert I, et al. Effectiveness of neonatal pulse oximetry screening for detection of critical congenital heart disease in daily clinical routine--results from a prospective multicenter study. Eur J Pediatr. 2010; 169:975.
10. Ewer AK, Middleton LJ, Furmston AT, et al. Pulse oximetry screening for congenital heart defects in newborn infants (PulseOx): a test accuracy study. Lancet. 2011; 378:785.
11. Thangaratinam S, Brown K, Zamora J, et al. Pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies: a systematic review and meta-analysis. Lancet. 2012; 379:2459.
12. Ewer AK, Furmston AT, Middleton LJ, et al. Pulse oximetry as a screening test for congenital heart defects in newborn infants: a test accuracy study with evaluation of acceptability and cost-effectiveness. Health Technol Assess 2012; 16:v.
13. Feltes T, Bacha E, Beekman R, et al. Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease. A Scientific Statement From the American Heart Association. Circulation. 2011;123:2607-2652