We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. [ncbi.nlm.nih.gov]

The deviation of the angle of the mouth to the left side, right-sided microphthalmia, with a small palpabral fissure, and right lateral rectus palsy were present. Bilateral rocker bottom feet were present. [jmgims.co.in]

More than 70 acute pediatric presentations are covered in a signs and symptoms section designed with side tabs for easy access. [books.google.de]

Patients with Eisenmenger Syndrome can present with: (i) Syncope This may be the first presenting feature in a child or young adult with pulmonary hypertension. [rcemlearning.co.uk]

• The size of the VSD affects the clinical presentation. 7. [slideshare.net]

• Coarctation of the Aorta

  LSE and/or mid left back ♥ ECG: RVH in 1st few months of life, LVH if older 26 27 Coarctation of the Aorta ♥ CXR: cardiomegaly evidence of CHF rib notching (older child) ♥ Treatment: surgery for ‘native’ CoA balloon angioplasty for re-CoA [slideplayer.com]

  The indications for intervention in coarctation of the aorta are significant hypertension and/or congestive heart failure along with a pressure gradient in excess of 20 mmHg across the coarctation; the type of intervention varies with age at presentation [link.springer.com]

  COARCTATION OF THE AORTA Narrowing of the aorta may occur anywhere along its length, but 98 percent of cases occur just below the origin of the left subclavian artery. 9 The classic clinical sign of coarctation of the aorta is a higher blood pressure [aafp.org]

• Asymptomatic

  3-5 years (earlier if symptomatic) or when diagnosed if later ♥ Two options for closure: surgery - suture or patch interventional catheter - device 15 Amplatzer ASD Occluder 16 PDA ♥ CHF symptoms if large ductus in very young infant, otherwise often asymptomatic [slideplayer.com]

  Infants may be asymptomatic or present with exercise intolerance, failure to thrive, and symptoms of heart failure. Load Less Saumil R Shah, Sergio Waxman, William H Gaasch, et al US Cardiology Review 2017;11(2):72–4. [radcliffecardiology.com]

  Clinical findings Patients are usually asymptomatic until complications occur (see below). [amboss.com]

  Mild MR may remain asymptomatic. Unless complicated by infective endocarditis, it may not progress further. Moderate and severe cases become symptomatic Atrial-Septal Defect (ASD). [pinterest.es]

• Anemia

  Iron deficiency anemia, known to affect bactericidal function, did not seem to affect the immune parameters in CCHD and ACHD groups. [ncbi.nlm.nih.gov]

  Paul Scott 149 Hematology Assessment 150 Anemia 151 Hemostatic Disorders 152 Blood Component Therapy Section 21 Oncology Thomas W. McLean and Marcia M. [maasmascotas.ga]

  Adequate oral iron intake prevents development of iron deficiency anemia. * After completion of the Fontan operation, specific dietary restrictions are not necessary. [rjmatthewsmd.com]

  Medical management of tetralogy of Fallot includes patient and parent education on ways to treat the “spells,” prevention of anemia and prophylaxis for subacute bacterial endocarditis. [aafp.org]

• Pallor

  • Hypoxic spells/ paroxysmal hypercyanotic attacks (1st 2years of life) – Severe hypoxia tissue acidosis breathlessness and pallor – Rapid increase in cyanosis – Restless and agitated – Inconsolable crying – An ambulatory toddler may squat – Severe [slideshare.net]

  * As the ductus arteriosus begins to close normally over the first 24-48 hours of life, symptoms of cyanosis, tachypnea, respiratory distress, pallor, lethargy, metabolic acidosis, and oliguria develop. [rjmatthewsmd.com]

  They are characterised by a rapid increase in cyanosis, usually associated with irritability or inconsolable crying because of severe hypoxia, and breathlessness and pallor due to tissue acidosis. [rcemlearning.co.uk]

• Pediatric Disease

  Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse [maasmascotas.ga]

• Failure to Thrive

  Infants may be asymptomatic or present with exercise intolerance, failure to thrive, and symptoms of heart failure. Load Less Saumil R Shah, Sergio Waxman, William H Gaasch, et al US Cardiology Review 2017;11(2):72–4. [radcliffecardiology.com]

  […] malnutrition can range from mild undernutrition to failure to thrive. [adc.bmj.com]

  […] to thrive sweating 7 VSD’s ♥ Exam (smaller VSD): pink normal pulses normal S1 and S2 ± systolic thrill harsh pansystolic murmur LLSE ♥ ECG: normal (smaller VSD) or LVH ± RVH (larger VSD) 8 VSD’s ♥ Larger defect: MDM @ apex (mitral flow murmur) narrowly [slideplayer.com]

  Infants may be asymptomatic or present with exercise intolerance, failure to thrive, and symptoms of heart failure. [amboss.com]

• Hepatomegaly

  Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory [ncbi.nlm.nih.gov]

  Signs of decompensation: Poor feeding Dyspnoea Hepatomegaly Engorged neck veins ↑pulse ↓pulse is a very poor prognostic sign! [almostadoctor.co.uk]

  Presentation: Cyanosis by the first days of life (50%) CHF within several weeks of life (30%) Single S2 Systolic murmur due to VSD or continuous murmur due to PDA CHF: hepatomegaly EKG Superior QRS axis Right atrial hypertrophy, left atrial hypertrophy [rcemlearning.co.uk]

  Clinical estimates of severity: VSD 82 Manish Chandra Prabhakar Clinical estimates of severity: PDA : Clinical estimates of severity: PDA 83 Manish Chandra Prabhakar RS examination : RS examination bilateral crepitations - CCF PA examination tender hepatomegaly [authorstream.com]

  […] ductus arteriosus, infants exhibit signs of cardiogenic shock, including the following: o Hypothermia o Tachycardia o Respiratory distress o Central cyanosis and pallor o Poor peripheral perfusion with weak pulses in all extremities and in the neck o Hepatomegaly [rjmatthewsmd.com]

• Heart Disease

  Implications of Rehabilitation Infants and toddlers with cyanotic congenital heart disease are likely at greater risk of gross motor delays and have a prolonged recovery period of gross motor ability compared to those with acyanotic congenital heart disease [ncbi.nlm.nih.gov]

  TABLE 7.12: ACYANOTIC CONGENITAL HEART DISEASE. In: Kahl LK, Hughes HK, eds. Harriet Lane Handbook. 21st ed. [unboundmedicine.com]

• Hypertension

  […] cp, cyanotic patients without pulmonary hypertension; and group cP, cyanotic patients with pulmonary hypertension. [adc.bmj.com]

  In some cases, the cause of the respiratory difficulty is compression of the tracheobronchial tree by hypertensive dilated pulmonary arteries, since this type of heart disease frequently demonstrates large left-to-right intracardiac shunts. [experts.umn.edu]

  hypertension, C/PH: Cyanotic patients with pulmonary hypertension. [scialert.net]

  […] and heart failure, but no cyanosis Eisenmenger syndrome Prolonged pulmonary hypertension due to a left-to-right shunt causes reactive constriction with permanent remodeling of pulmonary vessels → irreversible pulmonary hypertension Right ventricle hypertrophies [amboss.com]

• Cyanosis

  After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis. [ncbi.nlm.nih.gov]

  For the patients with PH and without cyanosis there was significant disparity in terms of all growth parameters including weight, height and head circumference as well, bone age compared to those with none of PH and cyanosis. [scialert.net]

  Assessing cyanosis When assessing whether cyanosis may be present, remember the following points: Peripheral cyanosis is normal in the first few days of life and cyanosis only whilst crying is rarely significant Polycythaemia or methaemoglobinaemia may [rcemlearning.co.uk]

  Clinical Manifestations • The degree of cyanosis depends on the amount of pulmonary stenosis. • Infants initially may be acyanotic. • Older children+ long standing cyanosis+ not undergone surgery – Dusky blue skin – Grey sclerae with engorged blood vessel [slideshare.net]

  Cyanosis is typically present where there is a right to left shunt Acyanotic disease is typically present when there is a left to right shunt – however – in pulmonary hypertension, the shunt can be reversed, and cyanosis can result. [almostadoctor.co.uk]

• Systolic Murmur

  […] thrill at LLSB and loud S 2 ± grade 3–4/6 holosystolic regurgitant murmur along LLSB ± systolic murmur of MR at apex ± mid-diastolic rumble at LLSB or at apex ± gallop rhythm Superior QRS axis RVH and LVH may be present Cardiomegaly with increased PVMs [unboundmedicine.com]

  […] aorta ♥ Treatment of valvar AS (moderate/severe): balloon valvuloplasty surgical valvotomy 25 Coarctation of the Aorta ♥ CHF in neonate if severe CoA; often asymptomatic in older child ♥ Exam: pink reduced or absent femoral pulses soft systolic murmur [slideplayer.com]

  BP 78/40, raised JVP. reduced ECG voltage, muffled heart sounds. mitral stenosis MS, is almost always due to what clinical presentation & examination of MS malar flush, fatigue, dyspnoea AF, tapping apex beat rumbling mid-diastolic murmur loud pan-systolic [cram.com]

  A PDA (IV prostaglandins) and Ionotropes (digoxin) What type of murmur is common with coarctation of the Aorta 4 defects associated with tetrology of fallot -VSD -Overriding aorta -Pulmonary stenosis => systolic murmur common -RVH "Tet" spell is associated [quizlet.com]

• 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]

  This can strain the heart and increase blood pressure in the lung arteries. A heart murmur might be the only sign of PDA. (A heart murmur is an extra or unusual sound heard during a heartbeat.) [medicinenet.com]

  While most heart murmurs in children are normal, some may be due to defects. If the heart problem is significant, your child’s pediatrician or family physician will likely refer your child to a pediatric cardiologist. [heart.org]

  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]

  Cyanotic heart disease (Medical Encyclopedia) Dextrocardia (Medical Encyclopedia) Echocardiogram -- children (Medical Encyclopedia) Heart murmurs and other sounds (Medical Encyclopedia) Patent ductus arteriosus (Medical Encyclopedia) Ventricular septal [icdlist.com]

• Seizure

  Fetus, and Newborn 59 Maternal Diseases Affecting the Newborn 60 Diseases of the Fetus 61 Respiratory Diseases of Newborn 62 Anemia and Hyperbilirubinemia 63 Necrotizing Enterocolitis 64 Hypoxic-Ischemic Encephalopathy, Intracranial Hemorrhage, and Seizures [maasmascotas.ga]

  Tetrology of Fallot -Restless/agitation -Inconsolable crying -Toddlers may Squat to compensate -Tachypnea with increasing cyanosis -Loss of consciousness, seizures, hemiparesis, or death if sever Treatment of Tetralogy of Fallot Tuck knees to chest, Morphine [quizlet.com]

  Slovenian type Hereditary dysplasia of blood vessel Lung agenesis with heart defect and thumb anomaly syndrome McKusick Kaufman syndrome Mental retardation, congenital heart disease, blepharophimosis, blepharoptosis and hypoplastic teeth Microcephaly, seizure [icdlist.com]

• Agitation

  Tetrology of Fallot -Restless/agitation -Inconsolable crying -Toddlers may Squat to compensate -Tachypnea with increasing cyanosis -Loss of consciousness, seizures, hemiparesis, or death if sever Treatment of Tetralogy of Fallot Tuck knees to chest, Morphine [quizlet.com]

  • Hypoxic spells/ paroxysmal hypercyanotic attacks (1st 2years of life) – Severe hypoxia tissue acidosis breathlessness and pallor – Rapid increase in cyanosis – Restless and agitated – Inconsolable crying – An ambulatory toddler may squat – Severe [slideshare.net]

  Occurs due to transient increase in right ventricular outflow tract obstruction that involvesnarrowing of the infundibulum (the sub-pulmonary outflow tract) Precipitating factors: Agitation and dehydration. [rcemlearning.co.uk]

• Anuria

  Adult Dose 20-80 mg IV/IM/PO up to tid Pediatric Dose 0.5-2 mg/kg IV/IM/PO up to tid Contraindications Documented hypersensitivity; hepatic coma, anuria, and severe electrolyte depletion Interactions Antagonizes muscle-relaxing effect of tubocurarine; [rjmatthewsmd.com]

  […] include: Reduced spontaneous movements Mottled skin Prolonged capillary refill time Decreased pulses or narrow pulse pressure Hypothermia and widening of toe-core temperature difference Tachycardia Tachypnoea and respiratory distress Hypotension Oliguria/ anuria [rcemlearning.co.uk]

• Oliguria

  Perfusion becomes poor, and metabolic acidosis and oliguria may develop. Coronary artery and cerebral perfusion also are dependent on systemic blood flow through the ductus arteriosus. [rjmatthewsmd.com]

  […] shock include: Reduced spontaneous movements Mottled skin Prolonged capillary refill time Decreased pulses or narrow pulse pressure Hypothermia and widening of toe-core temperature difference Tachycardia Tachypnoea and respiratory distress Hypotension Oliguria [rcemlearning.co.uk]

• Right Bundle Branch Block

  […] axis deviation; RAE, right atrial enlargement; RBBB, right bundle-branch block; RICS, right intercostal space; RUSB, right upper sternal border; RVH, right ventricular hypertrophy; SEM, systolic ejection murmur; VLBW, very low birth weight (i.e. [unboundmedicine.com]

  bundle branch block -secundum ASD – right axis deviation -partial AVSD – left axis deviation (superior axis) 20. [slideshare.net]

  Postoperative complications include conduction defects, such as AV conduction abnormalities and transient right bundle branch block. Atrial Septal Defect ASD is a defect in the septum dividing the right and left atria. [hawaii.edu]

  Clinical and Electrophysiologic Studies in Right Bundle Branch Block and Left Axis Deviation After Repair of Tetralogy of Fallot and Ventricular Septal Defects. 40th Annual Meeting of the American College of Chest Physicians. [sites.google.com]

  Postoperative complications include conduction defects, such as transient right bundle branch block. ATRIAL SEPTAL DEFECT Atrial septal defects may occur as sinus venosus, secundum or primum type. [aafp.org]

• Biventricular Hypertrophy

  AR, Aortic regurgitation; ASD, atrial septal defect; BP, blood pressure; BVH, biventricular hypertrophy; CDG, congenital disorders of glycosylation; CHD, congenital heart disease; CHF, congestive heart failure; HTN, hypertension; LAE, left atrial enlargement [unboundmedicine.com]

  • Small VSD: – Chest X-ray & ECG - normal • Large VSD:  Chest X-ray • Cardiomegaly • Enlarged pulmonary arteries • ↑ Pulmonary vascular markings • Pulmonary oedema  ECG : Biventricular hypertrophy and signs of pulmonary HPT  right ventricular enlargement [slideshare.net]

  […] the blood has circulated through the lungs) Loud pulmonary second sound (P2) from raised pulmonary arterial pressure Investigations Chest radiograph Cardiomegaly Enlarged pulmonary arteries Increased pulmonary vascular markings Pulmonary oedema ECG Biventricular [rcemlearning.co.uk]

[…] systolic thrill harsh pansystolic murmur LLSE ♥ ECG: normal (smaller VSD) or LVH ± RVH (larger VSD) 8 VSD’s ♥ Larger defect: MDM @ apex (mitral flow murmur) narrowly split S2 and loud P2 ± S3 CXR: cardiomegaly increased pulmonary vascularity 9 VSD’s ♥ Treatment [slideplayer.com]

Treatment surgery for native CoA balloon angioplasty for re-CoA [powershow.com]

Treatment:  Initial treatment (Medical) – diuretics and ACI (captopril) or digoxin diuretics for treatment of CHF.  Surgical repair of the entire defect ultimately is required. 26. Patent Ductus Arteriosus 27. [slideshare.net]

The treatment for a congenital heart defect depends on the type and severity of the defect. Some babies have mild heart defects that heal on their own with time. Others may have severe defects that require extensive treatment. [healthline.com]

Treatment for common types of congenital heart disease Specific treatments for the most common types of congenital heart disease are described below. [hse.ie]

Nutritional conciderations in the prognosis treatment of children with congenital heart disease. In: Suskind RM, Levinter-Suskind L., editors. Textbook of Pediatric Nutrition. [pediatricsciences.com]

The prognosis is usually bad, and the patient dies within the first five years of life. What are Acyanotic Congenital Heart Defects? Acyanotic congenital heart defects are also due to the inborn structural defects in the circulatory system. [differencebetween.com]

Prognosis The prognosis for children with congenital heart disease has improved dramatically over the last few decades. [patient.info]

Clinical Features, Complications, Prognosis And Treatment Of Mitral Regurgitation Disorder of mitral valve (MV) closure, either structural or functional, resulting in a backflow of part of the left ventricular (LV) stroke volume into the left atrium [pinterest.es]

It is crucial to be aware of congenital heart disease and tackle it early as in most cases the outcome and prognosis depend on whether the intervention is one at the appropriate time. [apollohospitals.com]

Altered oxygen content of the blood owing to hypoxia in CCHD patients may be an important etiological factor in the genesis of bacteremia and cerebral abscess. The affection of immune functions in ACHD cannot be adequately explained. [ncbi.nlm.nih.gov]

Subclavian artery supply disruption sequence: Hypothesis of vascular etiology for Poland, Klippel Feil and Möbius anomalies. Am J Med Genet 1986;23:903-18. [ PUBMED ] 7. Caravella L, Rogers GL. [jmgims.co.in]

References: [2] [3] [4] [5] [6] [7] [8] [9] Atrial septal defect (ASD) Etiology Down syndrome Fetal alchohol syndrome Holt-Oram syndrome Pathophysiology Impaired growth or excessive resorption of the atrial septa in utero leads to atrial septal defects [amboss.com]

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]

Etiology, Pathogenesis, Diagnosis and Treatment of Selected Congenital Heart Diseases. Department of Cardiac Physiopathology, State University of Palermo Medical School. Palermo, Italy. February 1988 70. [sites.google.com]

A large scare and population-based epidemiological investigation is warranted in future. [journals.plos.org]

Complications Heart failure in infancy Infective endocarditis Common cause of pulmonary hypertension and Eisenmenger syndrome in adolescents and adults References: [2] [3] [22] [23] [24] [25] Coarctation of the aorta Epidemiology Prevalence : 4/10,000 [amboss.com]

Cardiac abnormalities are the largest group of congenital defects Epidemiology 8 per 1000 children have a significant congenital cardiac abnormality 1 in 10 still births have a cardiac abnormality 10-15% of cases have more than one abnormality 10-15% [almostadoctor.co.uk]

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]

Pathophysiology General pathophysiological processes The left chambers of the heart are a high-pressure pump system and the right chambers of the heart are a low-pressure pump system. [amboss.com]

Sometimes the blueishness only happens when they’re working really hard, like feeding and crying (or thinking about the pathophysiological mechanisms of heart disease). [sketchymedicine.com]

Pathophysiology • VSD permits a left-to-right shunt to occur at the ventricular level with 3 adverse hemodynamic consequences: 1. left ventricular (LV) volume overload, 2. increased pulmonary blood flow, 3. compromise of systemic cardiac output. 9. [slideshare.net]

"Essentials of Pathophysiology: Concepts of Altered Health States" by Carol Porth says that congenital heart disease can be classified as cyanotic or acyanotic. [livestrong.com]

Some can also be used to prevent blood clots from forming or to control an irregular heartbeat. [healthline.com]

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]

Secundum ASDs • Prophylaxis for subacute bacterial endocarditis. • Surgical correction at 3-5 y/o to prevent right heart failure and arrhythmias in later life. Primum ASD 21. [slideshare.net]

A CAVC prevents oxygen-rich blood from going to the right places in your body. Your doctor can repair it with patches. But some people need more than one surgery to treat it. [webmd.com]

Preventive Cardiology - Fraz A. Ismat and Richard M. Donner Index [libreriacortinamilano.it]