cardiac Archives

cardiac

February 8 2022

Cardiac Causes of Cyanosis

PME-Admin article, medical cardiac, children, cyanosis, Heart Failure, infants, pediatrics, Tetralogy of Fallot, TGA, TOF, Transposition of the Great Arteries, Tricuspid Anomalies, Truncus Arteriosus

Cardiac Causes of Cyanosis in Newborn Infants — Santa J. Bartholomew M.D.

For newborns with congenital heart disease, early recognition and emergency stabilization and transport to a quaternary care pediatric center is essential. Cyanosis is a bluish color of the skin and tissues of a newborn infant. It occurs when the absolute level of reduced hemoglobin is more than 3-5 mg/dl. Cyanosis can be hard to evaluate in dark skinned infants making pulse oximetry in the newborn essential. Other physiologic factors can also affect cyanosis in the newborn such as hypothermia, high levels of fetal hemoglobin, alkalosis and low levels of 2,3 DPG (diphosphoglycerate).

Non-cardiac causes of cyanosis:*

Meconium aspiration in the newborn period
Newborn respiratory distress syndrome
Neonatal pneumonia
Pneumothorax
Diaphragmatic hernia and other disorders of the diaphragm, airway, or lung structures
Persistent pulmonary hypertension
Shock in the newborn
Acrocyanosis

* will be discussed in a separate article

Cardiac Causes as described by the acronym “The 6 T’s”

Transposition of the Great Arteries (TGA)
Tetralogy of Fallot (TOF)
Truncus Arteriosus (TA)
Total Anomalous Pulmonary Venous Connection (TAPVC)
Tricuspid Valve Anomalies (TA)
“Tons of other lesions” such as DORV (double outlet right ventricle), multiple variations of single ventricles, HLHS (hypoplastic left heart syndrome), syndromes associated with hetrotaxy syndrome and anomalous venous connections.

IMAGES OF LESIONS

D-GTA

TOF

Truncus Arteriosus Types

Tricuspid Anomalies

Total Anomalous Pulmonary Venous Connection (TAPVC)

Central cyanosis of the infant occurs when blood shunts right to left across the ductus arteriosus (PDA) which is the blood vessel used to oxygenate the infant inside the mother. In utero this vessel helps shunt blood away from the lungs and oxygenation of the infant occurs via the placenta. When the baby is born, and they take their first breath the pressure in the lungs falls and blood begins to flow to the lungs and the PDA begins to close. This closure does not occur completely for a couple of weeks after birth.

There are heart lesions that are so severe that they cause cyanosis of the newborn with the ductus arteriosus open, the cyanosis is independent of the patent ductus arteriosus. These include TAPVC, TA and TOF (may or may not be dependent on PDA). These children can be placed on oxygen in the nursery and have no increase in their oxygenation. In these newborns it is a medical emergency for them to be transported to a cardiac center to provide mixing between the left and right side of heart which must be done in cardiac catherization lab or in the operating room to avoid death.

Lesions that are ductal dependent mean that when the ductus arteriosus is open there is sufficient mixing of left and right heart that the infant can survive for short period of time if the duct remains open. These lesions include HLHS, Critical AS, CoA, interrupted aortic arch, TOF with pulmonary atresia, pulmonary atresia with intact ventricular septum.

Newborns with cyanotic heart lesions often have difficulty with feeding, feedings that take extremely long time or resting between sucking, frequent choking or gagging, fast breathing, poor weight gain, irritability, excessive sleeping, persistent pallor.

DIAGNOSTIC APPROACH

Physical examination: infants may have abnormal precordial activity, abnormal heart sounds, single or split-second heart sound and pathologic murmurs. In the abdomen they may have hepatosplenomegaly, diminished or absent pluses in the lower extremities and large variations in blood pressures between the arms and legs.
Universal Pulse Oximetry: right hand normal and all other extremities low.
CXR: cardiomegaly, abnormal cardiac silhouette, abnormal pulmonary markings.
Hyperoxia test: has not been used as much any more since ECHO is so available but putting the child on 100% and testing oximeter to see if oxygen saturation increases.

TREATMENT IN THE COMMUNITY HOSPITAL

When a blue infant is identified that was not identified on fetal ECHO hyperoxia test may be useful to determine cyanotic heart disease vs non-cardiac heart disease. When cyanotic heart disease is a concern then keeping the ductus arteriosus patent is essential. This can be done medically with the use of prostaglandin infusions while awaiting transport. It is essential to identify these infants before leaving the nursery or at the very latest at the 1-week visit. After the ductus closes many of these children will present in shock and their morbidity and mortality increases dramatically.

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January 6 2022

Chest Pain in Children

PME-Admin article, medical cardiac, chest pain, children, gastrointestinal, musculoskeletal, pediatrics, psychogenic, pulmonary

Chest Pain in Children — Santa J. Bartholomew M.D.

Chest pain in children and adolescents is a common complaint in both outpatient settings and in the emergency department. This is an anxiety provoking symptom for patients and their parents because of the fear from the adult literature of the cardiac origin of chest pain in adults. However, in children with chest pain the incidence of cardiac related causes is 0.2 to 1%. Most etiologies for chest pain in children are grouped into several categories:

Musculoskeletal (19.75%)
Pulmonary (8.39%)
Gastroenterologic (10.36%)
Psychogenic/Psychological (16.31%)
Cardiac (9.75%)
Idiopathic (36%) in some instances

Even though cardiac causes are very small etiologically they do and must take precedence in the evaluation of a child with chest pain because of the catastrophic consequences if missed. Determining the cause of chest pain relies heavily on a thorough history and physical examination.

ETIOLOGICAL CHEST PAIN CATEGORIES

MUSCULOSKELETAL

CAUSE	FEATURES OF PAIN	MANAGEMENT
Costochondritis: inflammation of costochondral junctions: ribs at jxn of sternum	Usually > 1 costochondral jxn. Reproducible pain on palpation	NSAIDS Supportive Care
Slipped Rib Syndrome: caused by subluxation of costal cartilage and nerve impingement. Usually 8,9,10th ribs, caused by trauma of the costal cartilages	Often involves ribs 8,9,10 Perception of slipping movement of ribs	Trigger avoidance Local nerve blocks
Teitz Syndrome: variant of costochondritis with visible swelling at junction with sternum.	Usually only 1 junction is affected and usually 1st or 2nd junction	NSAIDS Rest
Precordial Catch Syndrome: well localized, sharp, stabbing pain. Occurs at rest. Gets worse deep breathing	Etiology of pain unclear	Reassurance
Muscle Strain: usually from overuse or trauma	Common in pectoralis, intercostal muscles and serratus anterior. Usually h/o weightlifting, rowing. Can also be caused by severe coughing	NSAIDS Stretching Rest
Trauma: to chest wall	Usually rib fractures associated with bruising, +/-rib fractures	If assessment for other injuries negative: NSAIDS and rest

PULMONARY

CAUSE	FEATURES OF PAIN	MANAGEMENT
Asthma	A tightness described as pain	Treatment for asthma
Pluerodynia: sharp, spasmodic pain	Usually presents with fever and is caused by viral infection	NSAIDS, Tylenol Self-resolving
Infection: usually from cough	Associated findings on CXR	Appropriate treatment

GASTROINTESTINAL

CAUSE	FEATURES OF PAIN	MANAGEMENT
Gastroesophageal Reflux: usually burning pain	Epigastric pain, pain associated with regurgitation	Acid suppression
Esophagitis: burning pain	Secondary to reflux, allergy, or medications	Dx: via endoscopy Tx: control of reflux based on diagnosis
Foreign Body: common in children < 3 years old	Pain may be accompanied with drooling or unwillingness to swallow	Tx: endoscopy

CARDIAC

CAUSE	FEATURES OF PAIN	MANAGEMENT
Pericarditis	Usually, assoc. with fever and is immediately retrosternal. Cause often viral or idiopathic	Dx: ECG Tx: NSAIDS or ASA Steroids or colchicine in some cases
Myocarditis: usually viral (coxsackie B ,Parvo B19 and HSV 6)	Dx: ECHO and EKG	Supportive care
Mitral Valve Prolapse	Assoc. with mid to late systolic click with high-pitched late systolic murmur	Atbx prophylaxis Severe regurg may require valve replacement
Arrhythmia: especially SVT in children: narrow complex tachycardia	SVT V-Tach Can cause hypotension and ischemic chest pain and can degenerate into VF causing death	Hemodynamically stable: Vagal maneuvers, adenosine, identify and fix underlying issue Hemodynamically Unstable: direct current cardioversion Dx: ECG, ECHO, Troponins Tx: based on cause
Ischemia: caused by increased myocardial demand for blood and oxygen that cannot be met	Happens in: Left ventricular hypertrophy, Right ventricular hypertrophy Decreased myocardial supply: Kawasaki, cocaine use, thrombosis Congenital: Aberrant coronary artery, stenotic coronary orifice	Reassurance after thorough testing

PSYCHOGENIC

CAUSE	FEATURES OF PAIN	MANAGEMENT
Psychogenic	Prevalent in older children and adolescents. Assoc. with specific fears and recent stressors. 39% of adolescents are afraid of heart disorder or cancer	Reassurance after thorough testing

EVALUATION FOR CHEST PAIN

Very careful and complete history and physical examination: this will help guide the direction of testing. For each of the diseases listed there are often key historical features that can guide work-up.
There should be specific questioning about cardiac history, including personal and family history of syncope which may suggest cardiac issues. Family history regarding diabetes, connective tissue disorders like Marfan’s, Ehlers-Danlos, Kawasaki disease should be explored.
If cardiac etiology is suspected there is a standardized clinical assessment and management plan tool (SCAMP) that guides work up. Using this tool has provided a 22% cost reduction in evaluation and treatment of chest pain and to try to provide uniformity to evaluation.
When evaluating children with chest pain providers SHOULD ALWAYS consider conditions that may predispose children to cardiac death such as Long QT syndrome (20% of these patients have family history), hypertrophic cardiomyopathy (one third have family history), catecholaminergic polymorphic VT (VT brought on my exercise) and anomalous origin of the coronary artery.

QUESTIONS THAT SHOULD ALWAYS BE ASKED

Have you ever fainted, passed out, or had a seizure suddenly and without warning, especially during exercise or in response to auditory triggers such as doorbells, alarm clocks, and ringing telephones?
Have you ever had exercise-induced chest pain or shortness of breath?
Are you related to anyone with sudden, unexplained, and unexpected death before age 50 years?
Are you related to anyone who has been diagnosed as having a sudden death–predisposing heart condition such as hypertrophic cardiomyopathy, long QT syndrome, or catecholaminergic polymorphic ventricular tachycardia?

In evaluating a child with chest pain careful, methodical, and thorough history is key in guiding your evaluation!!