Infective endocarditis (IE) is defined as an infection of the endocardial surface of the heart.Fever, possibly low-grade and intermittent, is present in 90% of patients with IE. Heart murmurs are heard in approximately 85% of patients.One or more classic signs of IE are found in as many as 50% of patients. They include the following:- Petechiae: Common, but nonspecific, finding- Subungual (splinter) haemorrhages: Dark-red, linear lesions in the nail beds -Osler nodes: Tender subcutaneous nodules usually found on the distal pads of the digits- Janeway lesions: Nontender maculae on the palms and soles- Roth spots: Retinal haemorrhages with small, clear centres| rareSigns of neurologic disease, which occur in as many as 40% of patients, include the following:- Embolic stroke with focal neurologic deficits: The most common neurologic sign- Intracerebral haemorrhage- Multiple microabscessesOther signs of IE include the following:- Splenomegaly- Stiff neck- Delirium- Paralysis, hemiparesis, aphasia- Conjunctival haemorrhage- Pallor- Gallops- Rales- Cardiac arrhythmia- Pericardial rub- Pleural friction rub

A 3-dimensional transoesophageal echocardiography (3D TEE) provides direct visualization of the entire PFO anatomy and surrounding structures. It allows more accurate diagnosis.

The T wave represents ventricular repolarization. Other options:- P wave:Depolarization that spreads from the SA node throughout the atria. The wave lasts 0.08 to 0.1 seconds (80-100 ms).The isoelectric period after the P wave represents the time in which the impulse is travelling within the AV node.- P-R interval:Time from the onset of the P wave to the beginning of the QRS complex. The wave ranges from 0.12 to 0.20 seconds in duration.Represents the time between the onset of atrial depolarization and the onset of ventricular depolarization- QRS complex:It represents ventricular depolarization. The duration of the QRS complex is normally 0.06 to 0.1 seconds.- ST-segment:The isoelectric period following the QRS. It represents the period in which the entire ventricle is depolarized and roughly corresponds to the plateau phase of the ventricular action potential- U wave: It is a small positive wave which may follow the T wave. It represents the last remnants of ventricular repolarization.- Q-T intervalIt represents the time for both ventricular depolarization and repolarization to occur, and therefore roughly estimates the duration of an average ventricular action potential.The interval ranges from 0.2 to 0.4 seconds depending upon heart rate.At high heart rates, ventricular action potentials shorten in duration, which decreases the Q-T interval. Therefore the Q-T interval is expressed as a corrected Q-T (QTc) by taking the Q-T interval and dividing it by the square root of the R-R interval (interval between ventricular depolarizations). This allows an assessment of the Q-T interval that is independent of heart rate.The normal corrected Q-Tc interval is less than 0.44 seconds.

Kawasaki disease is an acute febrile illness of early childhood characterized by vasculitis of the medium-sized arteries. Diagnostic criteria include fever of at least 5 days duration along with 4 or 5 of the principal clinical features – extremity changes, polymorphous rash, oropharyngeal changes, conjunctival injection and unilateral nonpurulent cervical lymphadenopathy.

Although WPW syndrome is a type of supraventricular re-entrant tachycardia, CCBs are contraindicated in its management. This is because CCBs depress AVN conduction| the current then passes through the accessory pathway (bundle of Kent) instead, causing ventricular tachycardia.

DiGeorge syndrome is one of the most common microdeletion syndromes, resulting from 22q11 deletion. 10% of the cases can be inherited in an autosomal dominant fashion, while 90% are sporadic. The syndrome is characterized by a deficiency of both T and B-cell lines along with hearing loss, 20-fold increased lifetime chances of developing schizophrenia, renal abnormalities, congenital heart defects, and a borderline or low IQ. Distinctive facial features include micrognathia, long face, short philtrum, cleft palate, and small teeth.

The most common congenital cyanotic heart disease and the most common cause of blue baby syndrome, Tetralogy of Fallot shows four cardiac malformations occurring together. These are ventricular septal defect (VSD), pulmonary stenosis (right ventricular outflow obstruction), overriding aorta (degree of which is variable), and right ventricular hypertrophy. The primary determinant of severity of disease is the degree of pulmonary stenosis. Tetralogy of Fallot is seen in 3-6 per 10,000 births and is responsible for 5-7% congenital heart defects, with slightly higher incidence in males. It has also been associated with chromosome 22 deletions and DiGeorge syndrome. It gives rise to right-to-left shunt leading to poor oxygenation of blood. Primary symptom is low oxygen saturation in the blood with or without cyanosis at birth of within first year of life. Affected children ay develop acute severe cyanosis or ‘tet spells’ (sudden, marked increase in cyanosis, with syncope, and may result in hypoxic brain injury and death). Other symptoms include heart murmur, failure to gain weight, poor development, clubbing, dyspnoea on exertion and polycythaemia. Chest X-ray reveals characteristic coeur-en-sabot (boot-shaped) appearance of the heart. Treatment consists of immediate care for cyanotic spells and Blalock–Taussig shunt (BT shunt) followed by corrective surgery.

The most probable diagnosis for the patient in question is atrioventricular nodal re-entrant tachycardia (AVNRT).The presenting features of the patient are suggestive of supraventricular tachycardia (SVT). The most common form of SVT in children over 8 years old is atrioventricular nodal re-entrant tachycardia. It occurs as a result of an additional electrical circuit in or near the AV node itself.Other options:- Atrial Flutter: is a form of re-entry tachycardia within the atria which then becomes unsynchronised with the ventricles, which is not the case here. Furthermore, it is most common in babies and children with congenital heart disease.- Atrial tachycardia: is a condition where an area of atrium takes over the pacemaker activity of the heart. However, it is relatively uncommon.- Atrioventricular re-entrant tachycardia (AVRT): occurs due to an accessory electrical pathway between the atria and ventricles forming a re-entry circuit. This is the most common cause of SVT in children < 8 years old. One of the specific diagnoses of AVRT is Wolff-Parkinson-White syndrome. However, WPW syndrome is much rarer than AVNRT.- Premature atrial contractions (PACs): are very common in normal, healthy children and adolescents. Occasionally, they can give rise to the feeling of a 'skipped beat'. Fortunately, they are rarely associated with any significant underlying pathology or need to be treated.

The murmur and thrill indicate a right outflow tract murmur, coupled with the right ventricular heave suggests right ventricular hypertrophy. The VSD would not have been amenable to surgery due to the child’s size/weight and so instead would have been palliated with a PA band. As the child grows, this band progressively restricts pulmonary blood flow until the left to right shunt has reversed. Then it is time for the band to be removed and the VSD to be closed.Pulmonary atresia is a cyanotic condition, which may be palliated with an arterial duct stent. This answer is incorrect as the stent placement would not require a midline sternotomy and would give a continuous machinery type murmur. Pulmonary stenosis with PDA ligation is incorrect. The murmur, thrill, and heave all match a pulmonary stenosis but in the absence of an additional shunt lesion the child would not be cyanosed.

Cyanotic congenital heart disease (CCHD) is a common cause of neonatal morbidity and mortality. They can be classified as CCHD due to:- Right-to-left shunt, associated with the decreased pulmonary flow, e.g., tetralogy of Fallot (TOF), pulmonary atresia, right-sided hypoplastic heart,- Right-to-left shunt, associated with the decreased aortic flow, e.g., left-sided hypoplastic heart, interrupted arch, severe coarctation| – Bidirectional shunt, e.g., TGA, DORV, TA, etc. Eisenmenger syndrome is not a neonatal CCHD| rather it develops later in young adulthood secondary to various CHD.

Except Tetralogy of Fallot, the others are acyanotic heart diseases. The cyanotic spells and loss of consciousness are due to spasm of the infundibular septum, which acutely worsens the right ventricular outflow tract obstruction. The given murmur is due to a ventricular septal defect.

Marfan syndrome is a disorder that affects the connective tissue found throughout the body, Marfan syndrome can affect many systems, often causing abnormalities in the heart, blood vessels, eyes, bones, and joints. The two primary features of Marfan syndrome are vision problems caused by a dislocated lens (ectopia lentis) in one or both eyes and aortic root disease, leading to aneurysmal dilatation, aortic regurgitation and dissection is the main cause of morbidity and mortality in Marfan syndrome.

The murmur of tricuspid valve regurgitation is typically a high-pitched, blowing, holosystolic, plateau, nonradiating murmur best heard at the lower left sternal border. The intensity is variable, but tends to increase during inspiration (Carvallo’s sign), with passive leg raising, after a post-extrasystole pause, and following amyl nitrite inhalation. The intensity of the murmur tends to correlate positively with the severity of regurgitation. Right ventricular enlargement may displace the location of the murmur leftward. Right ventricular failure may abolish respiratory variation. When tricuspid regurgitation is caused by pulmonary hypertension, a pulmonic ejection click may be audible. Severe tricuspid regurgitation is commonly accompanied by a third heart sound emanating from the right ventricle and best heard at the lower left sternal border. Severe tricuspid regurgitation typically produces an accentuated jugular cv wave and may produce hepatic congestion with a pulsatile liver.The auscultatory findings associated with ventricular septal defect are variable, depending on a variety of morphologic and hemodynamic considerations. The systolic murmur associated with a Roger’s-type ventricular septal defect (regurgitant jet flows directly into the right ventricular outflow tract) in patients with low pulmonary vascular resistance is a low to medium pitched, holosystolic murmur with midsystolic accentuation. The intensity of the murmur is typically grade 3 or higher.Patent ductus arteriosus produces a continuous murmur in patients with normal pulmonary vascular resistance.

Ebstein’s anomaly is characterised by apical displacement and adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium, thereby displacing the functional tricuspid orifice apically and dividing the right ventricle into two portions. The main haemodynamic abnormality leading to symptoms is tricuspid valve incompetence. The clinical spectrum is broad| patients may be asymptomatic or experience right-sided heart failure, cyanosis, arrhythmias and sudden cardiac death (SCD). Many Ebstein’s anomaly patients have an interatrial communication (secundum atrial septal defect (ASD II) or patent foramen ovale). Other structural anomalies may also be present, including a bicuspid aortic valve (BAV), ventricular septal defect (VSD), and pulmonary stenosis. The morphology of the tricuspid valve in Ebstein anomaly, and consequently the clinical presentation, is highly variable. The tricuspid valve leaflets demonstrate variable degrees of failed delamination (separation of the valve tissue from the myocardium) with fibrous attachments to the right ventricular endocardium.The displacement of annular attachments of septal and posterior (inferior) leaflets into the right ventricle toward the apex and right ventricular outflow tract is the hallmark finding of Ebstein anomaly.

A ventricular septal defect (VSD) is a common form of congenital heart defects and is characterised by the presence of a hole in the wall that separates the right from the left ventricle. Medium or large defects can cause many complications. One of these complication is Eisenmenger syndrome, characterised by reversal of the shunt (from left-to-right shunt into a right-to-left) ,cyanosis and pulmonary hypertension.

The most probable diagnosis for this child would be rheumatic fever due to a previous history of rheumatic fever, evidence of streptococcal disease from a throat swab, a raised ASO titre and a positive streptococcal antigen test or a leukocytosis.Acute Rheumatic Fever:ARF occurs because of an abnormal immune response to a streptococcal antigenic component. It has a latent period of 1–3 weeks and is more common in the lower socio-economic classes. It peaks at around 5–15 years of age and affects the blood vessels, joints, nervous system and subcutaneous tissues. It is characterised as an autoimmune disease, and there is a risk of rheumatic fever occurring after infection in 3% of the population. The recurrence is greater in younger children and increases with each attack. Duckett-Jones criteria:The diagnostic criteria for acute rheumatic fever.Major: – Pancarditis- Chorea (Sydenham’s) – Polyarthritis (flitting)- Erythema marginatum- Subcutaneous nodulesMinor criteria include the presence of arthralgia, fever, prolonged PR interval, raised ESR, raised CRP.Note that:To make the diagnosis of rheumatic fever: Two major and/or one major with two minor criteria are required. Evidence of a recent streptococcal infection with a raised ASO titre or an antiribonuclease B level is sufficient. Exceptions to this rule are mentioned below:- Chorea alone is diagnostic.- Insidious or late-onset carditis with no other explanation.- Rheumatic recurrence: The presence of one major and one minor criterion with a prior streptococcal disease that is recurring.Consequences of pericarditis include heart block, pericardial effusion, tachycardia, cardiomegaly, pericardial friction rub, congestive cardiac failure, valvular disease and a Carey–Coombes apical mid-diastolic rumbling murmur.New heart murmurs are often audible, including those of mitral regurgitation and aortic regurgitation. Skin nodules affect the perivascular tissues and are non-specific lesions resulting from fibroid degeneration.Management:Medication includes aspirin for the acute phase, non-steroidal anti-inflammatory drugs for arthritis, prednisolone for severe carditis, and high-dose penicillin for immediate management with antibiotic prophylaxis in the long term. Antibiotics may include penicillin V, erythromycin or benzylpenicillin. Diazepam and haloperidol may be required to control the chorea.

The most common congenital cyanotic heart disease and the most common cause of blue baby syndrome, Tetralogy of Fallot shows four cardiac malformations occurring together. These are ventricular septal defect (VSD), pulmonary stenosis (right ventricular outflow obstruction), overriding aorta (degree of which is variable), and right ventricular hypertrophy. The primary determinant of severity of disease is the degree of pulmonary stenosis. Tetralogy of Fallot is seen in 3-6 per 10,000 births and is responsible for 5-7% congenital heart defects, with slightly higher incidence in males. It has also been associated with chromosome 22 deletions and DiGeorge syndrome. It gives rise to right-to-left shunt leading to poor oxygenation of blood. Primary symptom is low oxygen saturation in the blood with or without cyanosis at birth of within first year of life. Affected children ay develop acute severe cyanosis or ‘tet spells’ (sudden, marked increase in cyanosis, with syncope, and may result in hypoxic brain injury and death). Other symptoms include heart murmur, failure to gain weight, poor development, clubbing, dyspnoea on exertion and polycythaemia. Chest X-ray reveals characteristic coeur-en-sabot (boot-shaped) appearance of the heart. Treatment consists of immediate care for cyanotic spells and Blalock–Taussig shunt (BT shunt) followed by corrective surgery.

Auscultatory findings of VSD vary with the size of the defect. Small VSDs typically produce murmurs ranging from a grade 1 to 2/6 high-pitched, short systolic murmur (due to tiny defects that actually close during late systole) to a grade 3 to 4/6 holosystolic murmur (with or without thrill) at the lower left sternal border| this murmur is usually audible within the first few days of life (see table Heart Murmur Intensity). The precordium is not hyperactive, and the 2nd heart sound (S2) is normally split and has normal intensity.Moderate to large VSDs produce a holosystolic murmur that is present by age 2 to 3 wk| S2 is usually narrowly split with an accentuated pulmonary component. An apical diastolic rumble (due to increased flow through the mitral valve) and findings of heart failure (e.g., tachypnoea, dyspnoea with feeding, failure to thrive, gallop, crackles, hepatomegaly) may be present. In moderate, high-flow VSDs, the murmur is often very loud and accompanied by a thrill (grade 4 or 5 murmur). With large defects allowing equalization of left ventricular and right ventricular pressures, the systolic murmur is often attenuated.

Pulmonary arterial hypertension (PH) is noted in 9 to 35 % of patient with a secundum type ASD. The haemodynamic definition of PH is a mean pulmonary arterial pressure (mPAP) of ≥25 mmHg at rest by means of right heart catheterization. In ASD, The findings on physical examination depend on the degree of left-to-right shunt and its hemodynamic consequences, which, in turn, depends on the size of the defect, the diastolic properties of both ventricles, and the relative resistance of the pulmonary and systemic circulations.Blood flow across the atrial septal defect (ASD) does not cause a murmur at the site of the shunt because no substantial pressure gradient exists between the atria. However, ASD with moderate-to-large left-to-right shunts results in increased right ventricular stroke volume across the pulmonary outflow tract creating a crescendo-decrescendo systolic ejection murmur. This murmur is heard in the second intercostal space at the upper left sternal border.Patients with large left-to-right shunts often have a rumbling mid-diastolic murmur at the lower left sternal border because of increased flow across the tricuspid valve.ASD is an acyanotic lesion. Thus, the patient should be normally saturated. In the rare case of severe pulmonary arterial hypertension, atrial shunt reversal (Eisenmenger syndrome) may occur, leading to cyanosis and clubbing

The most probable diagnosis in this patient would be pulmonary stenosis. Pulmonary Stenosis:Pulmonary valve murmurs are heard in the upper left sternal edge, associated with a thrill but no desaturation in the absence of an additional shunt. The ECG changes suggest right ventricular hypertrophy. Pulmonary stenosis is often well tolerated in childhood unless severe. These should be monitored with serial echocardiography, and balloon pulmonary valvoplasty should be considered once the pressure gradient reaches 64 mmHg.Other options:- Atrial septal defect: While atrial septal defects are associated with right ventricular outflow tract murmurs, they would not cause a thrill.- Patent ductus arteriosus: PDA murmurs can be audible in the left upper sternal edge, but would normally be audible in the left infraclavicular area and be continuous rather than ejection systolic. This left to right shunt would not cause desaturation but does cause left-sided volume loading and hence left-sided ECG changes.- Tetralogy of Fallot: Ventricular septal defect, overriding aorta, subpulmonary stenosis, and right ventricular hypertrophy. This would often cause a ULSE murmur with a thrill and RVH on ECG. However, this degree of obstruction would cause shunting from right to left, and this child would be desaturated.- Ventricular septal defect: Isolated ventricular septal defects cause pansystolic murmurs at the left lower sternal edge. They would have left-sided ECG changes and normal saturations (in the absence of pulmonary hypertension).