Pediatric Cardiovascular Disorders

Match each patient's clinical vignette with the most appropriate initial targeted intervention.

Match the following classic pediatric clinical presentations and physical examination findings with their most likely underlying congenital heart disorder.

Match each patient's clinical presentation (indicating a specific genetic syndrome or teratogenic exposure) with the congenital heart defect most classically associated with their condition.

Match each clinical presentation of a pediatric cardiovascular disorder to its precise underlying embryologic or pathophysiological mechanism.

A 2-month-old girl is brought to the emergency department by her parents due to a 3-day history of rapid breathing, poor feeding, and severe irritability. The parents report that the infant becomes completely drenched in sweat, turns pale, and cries inconsolably about 5 minutes into every bottle feeding. She was born at 39 weeks gestation and had an uncomplicated newborn nursery course. Her weight has dropped from the 50th percentile at birth to the 5th percentile currently. Her temperature is 36.9°C (98.4°F), heart rate is 178/min, respiratory rate is 68/min, and blood pressure is 74/45 mm Hg. Pulse oximetry is 96% on room air. Physical examination shows intercostal retractions, nasal flaring, and diffuse expiratory wheezes bilaterally. Cardiac auscultation reveals a prominent S3 gallop and a grade 2/6 holosystolic murmur at the apex. The liver edge is palpable 4 cm below the right costal margin. Femoral and brachial pulses are equal and symmetric. Chest radiography demonstrates severe cardiomegaly and increased interstitial markings. Which of the following is the most appropriate next step in the management of this patient?

A $6$-day-old male neonate is evaluated in the emergency department due to poor feeding, lethargy, and increased work of breathing over the past $12$ hours. He was born at $39$ weeks gestation via an uncomplicated vaginal delivery and was discharged home at $48$ hours of life. Temperature is $36.4^\circ\text{C}$ ($97.5^\circ\text{F}$), heart rate is $185$/min, and respiratory rate is $68$/min. Oxygen saturation is $94\%$ on room air in the right hand; a reliable plethysmographic waveform cannot be obtained in either foot. Physical examination reveals an ashen, mottled appearance with a prolonged capillary refill time of $5$ seconds. Marked subcostal retractions and grunting are present. The right brachial pulse is full and bounding, while bilateral femoral pulses are barely palpable. Blood pressure measured in the right arm is $84/45$ mmHg, and in the left leg is $46/24$ mmHg. Arterial blood gas analysis reveals a pH of $7.12$, pCO $_2$ of $32$ mmHg, and pO $_2$ of $75$ mmHg, with a calculated bicarbonate of $10$ mEq/L. Which of the following is the most appropriate initial step in management?

A 15-month-old boy with a known, unrepaired congenital heart defect is brought to the pediatric cardiology clinic for a follow-up evaluation. The parents report that earlier today, the child became extremely fussy, began breathing rapidly, and developed a deep bluish discoloration around his lips during a crying spell. The mother immediately placed the toddler on his back and pushed his knees firmly against his chest, which rapidly resolved the cyanosis and calmed him down. This physical maneuver improved the patient's condition primarily by causing which of the following physiologic changes?

An $8$-week-old female infant presents to the urgent care clinic with fussiness and refusal to feed for the past four hours. The infant is alert and responsive. Vital signs include a heart rate of $270\text{ /min}$, respiratory rate of $50\text{ /min}$, and blood pressure of $82/48\text{ mmHg}$. Peripheral pulses are robust, capillary refill is under $2\text{ seconds}$, and extremities are warm. A 12-lead electrocardiogram shows a regular, narrow-complex tachycardia with absent P waves and constant R-R intervals. A peripheral intravenous line is already in place. Arrange the following acute management steps in the correct progressive order, from the initial non-pharmacologic approach to subsequent therapies if the arrhythmia persists.

A $12$-day-old male infant is brought to the emergency department by his parents due to lethargy, poor feeding, and rapid breathing that started earlier today. He was born at $39$ weeks gestation via an uncomplicated spontaneous vaginal delivery and was discharged on day $2$ of life. The prenatal course was unremarkable. His temperature is $36.8^\circ\text{C}$ ($98.2^\circ\text{F}$), heart rate is $185/\text{min}$, respiratory rate is $72/\text{min}$, and oxygen saturation is $94\%$ on room air. Blood pressure is $75/45\text{ mmHg}$ in the right arm and $50/30\text{ mmHg}$ in the left leg. On physical examination, the infant appears pale and mottled. Capillary refill time is $4$ seconds. Brachial pulses are bounding, but femoral pulses are barely palpable bilaterally. A grade $2/6$ systolic ejection murmur is heard at the left sternal border. Lung auscultation reveals mild bilateral crackles. Arterial blood gas analysis shows a pH of $7.15$, $p\text{CO}_2$ of $32\text{ mmHg}$, and $\text{HCO}_3^-$ of $11\text{ mEq/L}$. Which of the following is the most appropriate initial pharmacological intervention for this patient?

A 5-day-old female neonate, born prematurely at 28 weeks gestation, is evaluated in the neonatal intensive care unit for increasing respiratory distress. Over the past 12 hours, she has developed worsening tachypnea and an increased need for supplemental oxygen. Vital signs show a heart rate of 168/min, respiratory rate of 72/min, and blood pressure of 55/25 mm Hg. Physical examination reveals bounding peripheral pulses in all four extremities and a hyperactive precordium. Cardiac auscultation demonstrates a prominent, continuous, "machine-like" murmur heard best at the left second intercostal space. Echocardiography confirms a patent vascular connection between the descending aorta and the main pulmonary artery with left-to-right shunting. Which of the following is the most appropriate initial pharmacologic intervention to close this defect?

Match each common pediatric congenital cardiovascular disorder with its classic physical examination finding.

Match each pediatric patient's clinical presentation with the most likely congenital cardiac anomaly.

A 2-year-old boy is brought to the pediatric emergency department with a 6-day history of high fever, bilateral non-exudative conjunctivitis, cracked red lips, and a polymorphous maculopapular rash on his trunk. Physical examination reveals cervical lymphadenopathy and erythema of the palms and soles. A clinical diagnosis of Kawasaki disease is made. Arrange the following steps in the correct chronological sequence for the standard management and follow-up of this patient.

A 5-month-old boy is brought to the emergency department by his parents due to a persistent fever for the past 6 days. His mother reports he has been extremely irritable, feeding poorly, and has a mild, dry cough. A transient erythematous macular rash was noted on his trunk 3 days ago but has since resolved. His temperature is 39.8°C (103.6°F), heart rate is 175/min, blood pressure is 85/50 mmHg, and respiratory rate is 45/min. On physical examination, the infant is fussy but consolable. He has bilateral non-exudative conjunctival injection and erythematous, cracked lips. There is no cervical lymphadenopathy, and his hands and feet appear normal without edema or peeling. Breath sounds are clear bilaterally. Cardiac examination reveals a tachycardic, regular rhythm with no murmurs. Laboratory studies reveal:

Urinalysis demonstrates 25 leukocytes/hpf, negative nitrites, and no bacteria. Which of the following is the most appropriate next step in the management of this patient?

A 4-month-old boy is brought to the emergency department in January with a 3-day history of rapid breathing, poor feeding, and excessive diaphoresis during feeds. He was noted to have a 'heart murmur' at birth but has not been evaluated by a cardiologist. On physical examination, his temperature is 37.2°C (99.0°F), heart rate is 168/min, respiratory rate is 65/min, and blood pressure is 85/50 mm Hg in the right arm. Oxygen saturation is 94% on room air. Femoral pulses are 2+ and equal bilaterally, with no brachial-femoral delay. Lung auscultation reveals diffuse bilateral expiratory wheezes and fine basilar crackles. Cardiac examination demonstrates a hyperactive precordium, a 4/6 holosystolic murmur heard best at the left lower sternal border, and a prominent S3 gallop. The liver edge is palpable 4 cm below the right costal margin. Suspecting a severe respiratory infection, a resident physician administers nebulized albuterol and oral dexamethasone. Thirty minutes later, the infant becomes increasingly lethargic, his heart rate rises to 215/min, and his respiratory distress significantly worsens. Which of the following best describes the underlying pathophysiology of this patient's initial presentation, and why did the administered pharmacotherapy exacerbate his clinical deterioration?

An 8-week-old girl is evaluated in the pediatric clinic for poor weight gain and respiratory difficulty. Her parents report that over the past three weeks, she has become increasingly irritable during feedings, sweats profusely, and frequently pauses to catch her breath. She has gained only 90 grams since her 1-month well-child visit. Her temperature is 37.0°C (98.6°F), heart rate is 166/min, respiratory rate is 65/min, and blood pressure is 85/50 mm Hg. Pulse oximetry shows 97% on room air. Physical examination reveals intercostal retractions and nasal flaring. Auscultation of the lungs demonstrates scattered bilateral wheezes and mild bibasilar crackles. Cardiac examination reveals a hyperdynamic precordium, a palpable thrill, and a harsh, 4/6 holosystolic murmur heard best at the left lower sternal border. The liver edge is palpated 3.5 cm below the right costal margin. Which of the following is the most appropriate next step in management?

Match each clinical presentation and combination of diagnostic findings to the most likely congenital heart defect.

Match each pediatric patient's clinical presentation with the congenital cardiovascular anomaly most likely to be found on echocardiography.

A 14-month-old girl is rushed to the pediatric emergency center by her parents because she suddenly became severely cyanotic and limp after a prolonged crying episode at home. On arrival, she is highly agitated, inconsolable, and instinctively drawing her legs toward her abdomen. Vital signs show a heart rate of 178/min, respiratory rate of 52/min, and oxygen saturation of 62% on room air. Auscultation reveals a harsh, grade 3/6 systolic ejection murmur at the left mid-to-upper sternal border, which the parents note is much softer than what was described during her last pediatric visit. She is diagnosed with a hypercyanotic spell secondary to an underlying congenital heart defect. Arrange the following interventions in the most appropriate sequential order for the acute escalation of management for this patient, starting with the immediate first step and progressing to treatments for medically refractory symptoms.

An 8-month-old infant is rapidly triaged in the emergency department following a syncopal event at home. According to the parents, the child was crying vigorously during a diaper change when he suddenly became deeply blue around the lips and face, followed by a brief loss of consciousness. The mother notes he has had similar, though milder, episodes of blueness when agitated over the past few weeks. The infant was born at a rural clinic and has not received subsequent medical care. On physical examination, the infant is awake but severely agitated. Vital signs demonstrate a heart rate of 180/min, respiratory rate of 55/min, and oxygen saturation of 74% on room air. Auscultation reveals a prominent right ventricular impulse and a harsh, crescendo-decrescendo systolic murmur at the left upper sternal border. Lung fields are clear to auscultation. Which of the following is the most appropriate initial step in the management of this patient?