Genetic Link to Unusual Cardiac Diagnosis
Genetic Link to Unusual Cardiac Diagnosis
This is a Critical Care Critical Thinking Problem (CCCTP) designed to test your problem solving and analysis abilities.
Instructions: Read the CCCTP below. Then outline how you would assess and expect this patient to be managed. Finally compare your rationale and decision to that provided in the shaded area.
We invite your contributions to this section of the journal. An honorarium of $50 will be paid to the author of a published CCCTP.
Please submit material to: Beth Suddaby; Inova Fairfax Hospital for Children; 3300 Gallows Rd.; Falls Church, VA 22042 or e-mail at elizabeth.suddaby@inova.com for author guidelines.
John was a 2-hour-old, apparently healthy, newborn boy who had an episode of duskiness in the newborn nursery and was assessed by the neonatologist on call. The neonatologist found diminished pulses and lower extremities cooler than upper. The infant was transferred to the neonatal intensive care unit (NICU).
John was the first-born child of parents who were in their twenties. He was delivered vaginally at full term weighing 3.4 kg. His mother was group B strep positive. All other prenatal testing was negative for any problems with mother or baby.
General Appearance: John was well developed with no abnormal facies or features.
Vital Signs: HR - 170; RR - 54; Temp. - 98.4; BP on right arm 64/40; BP on leg was unable to be obtained; O2 saturation 99%.
Neuromuscular: Vigorous, active baby who resisted medical procedures.
Respiratory: Lungs clear to ausculatation.
Cardiovascular: Skin was cool to touch, particularly on distal extremities. Capillary filling time was extended at 4 seconds; brachial pulses were 1+; femoral pulses were intermittently 1+; but distal pulses were difficult to palpate.
GI/GU: Normal external genitalia, no urine output since birth, abdomen soft and not distended.
With questionable cardiac findings of diminished pulses and one episode of cyanosis, a cardiology consult was obtained and echocardiogram ordered. In light of the history of group B strep exposure, antibiotics were also started after a peripheral IV was placed.
Prior to the echo technologist arriving to obtain more clinical information, John had another episode of desaturation that was observed by the NICU nurse and neonatalogist who was called to the bedside. John was crying and changed first from red to more bluish color and then became limp and gray. The pulse oximeter on his right hand that had been reading 99% did not decline but just stopped altogether (no waveform, no measurement). While he was limp and gray, John also had a period of apnea.
Because of this episode, John was intubated and started on a prostaglandin infusion at 0.1 mcg/kg/min. Umbilical arterial and venous lines were placed for monitoring. The episode was described to the pediatric cardiologist who accompanied the echo technologist to the bedside. What do you expectthe diagnosis to be when they complete this echo?
This is a Critical Care Critical Thinking Problem (CCCTP) designed to test your problem solving and analysis abilities.
Instructions: Read the CCCTP below. Then outline how you would assess and expect this patient to be managed. Finally compare your rationale and decision to that provided in the shaded area.
We invite your contributions to this section of the journal. An honorarium of $50 will be paid to the author of a published CCCTP.
Please submit material to: Beth Suddaby; Inova Fairfax Hospital for Children; 3300 Gallows Rd.; Falls Church, VA 22042 or e-mail at elizabeth.suddaby@inova.com for author guidelines.
John was a 2-hour-old, apparently healthy, newborn boy who had an episode of duskiness in the newborn nursery and was assessed by the neonatologist on call. The neonatologist found diminished pulses and lower extremities cooler than upper. The infant was transferred to the neonatal intensive care unit (NICU).
John was the first-born child of parents who were in their twenties. He was delivered vaginally at full term weighing 3.4 kg. His mother was group B strep positive. All other prenatal testing was negative for any problems with mother or baby.
General Appearance: John was well developed with no abnormal facies or features.
Vital Signs: HR - 170; RR - 54; Temp. - 98.4; BP on right arm 64/40; BP on leg was unable to be obtained; O2 saturation 99%.
Neuromuscular: Vigorous, active baby who resisted medical procedures.
Respiratory: Lungs clear to ausculatation.
Cardiovascular: Skin was cool to touch, particularly on distal extremities. Capillary filling time was extended at 4 seconds; brachial pulses were 1+; femoral pulses were intermittently 1+; but distal pulses were difficult to palpate.
GI/GU: Normal external genitalia, no urine output since birth, abdomen soft and not distended.
With questionable cardiac findings of diminished pulses and one episode of cyanosis, a cardiology consult was obtained and echocardiogram ordered. In light of the history of group B strep exposure, antibiotics were also started after a peripheral IV was placed.
Prior to the echo technologist arriving to obtain more clinical information, John had another episode of desaturation that was observed by the NICU nurse and neonatalogist who was called to the bedside. John was crying and changed first from red to more bluish color and then became limp and gray. The pulse oximeter on his right hand that had been reading 99% did not decline but just stopped altogether (no waveform, no measurement). While he was limp and gray, John also had a period of apnea.
Because of this episode, John was intubated and started on a prostaglandin infusion at 0.1 mcg/kg/min. Umbilical arterial and venous lines were placed for monitoring. The episode was described to the pediatric cardiologist who accompanied the echo technologist to the bedside. What do you expectthe diagnosis to be when they complete this echo?
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