Congenital heart murmurs
Low arterial PO2 - Pt presents w/ Central cyanosis - Central cyanosis most likely presents from: -A) Etiology ----1) Congenital heart disease -B) Signs/Symptoms ----1) Tachypnea & Diaphoresis → while breastfeeding --------Highly suggestive of Congenital heart disease Polycythemia - Neonatal polycythemia is a consequence of GDM or prematurity - It can present w/ cyanosis - However, key differences include: ---1) Cyanosis in polycythemia presents as a PERIPHERAL CYANOSIS ------[pt has CENTRAL cyanosis] ---1) Must have a HCT of ≥65% -----[pt has no accompanying labs to Dx neonatal polycythemia] ---2) Usually presents w/ accompanying respiratory distress ------[Pt has NO respiratory distress] ---3) Usually presents w/in the FIRST FEW DAYS OF LIFE -------[pt is 6 WEEKS OLD]
(UWS2E1) A 6-week-old AA male appears to breath rapidly & becomes diaphoretic after breast feeding for 10-15 mins. Exam reveals bluish discoloration of his nail beds, lips, & tongue. His extremities are warm. Which of the following is the most likely cause of his physical findings? (Polycythemia VS Low Arterial O2)
No intervention - Pt presents w/ a patent foramen ovale - Presentation incldues: --1) Transient right-to-left inter-atrial shunt that becomes MORE APPARENT W/ COUGH --2) Murmur ----Soft mid-diastolic murmur best heard at left lower sternal border MGMT includes: --1) No further diagnostics/TMT ------Diagnostics/TMT only necessary when paradoxical embolism occurs Surgical closure - Only indicated in large PDAs (> 2.5 cm)
A 19-month-old girl is brought for a well-child examination. She was born at term and has been healthy. On physical examination, the child is alert and active. Her temperature is 37.3 °C (99.1 °F), pulse is 102/min, respirations are 24/min, and blood pressure is 102/54 mm Hg. She is at the 75th percentile for height and 80th percentile for weight. Cardiac examination shows a normal S1 and a split S2 during inspiration. A grade 2/6 soft mid-systolic murmur is heard maximally at the lower left sternal border. A contrast echocardiogram is performed and reveals a mild transient right-to-left interatrial shunt that becomes apparent when the child coughs. Which of the following is the most appropriate next step in management? (No intervention OR Surgical closure)
Outpatient follow-up - MGMT of VSD is based on size & symptoms --a) Small, asymptomatic VSDs -----Observation & follow-up → usually spontaneous resolve -----A small VSD is LOUD (5/6 murmur), a large VSD would present as a 1/6 or 2/6 murmur --b) Evaluation & diuretics ----(if symptoms arise: tachypnea, failure to thrive, distress during feeding) --c) Surgery ----Pts who still have symptoms or worsen (pulm HTN, LV dilation) despite medical therapy
A 2-week-old newborn is brought to the physician for a follow-up examination. He was born at term and the pregnancy was uncomplicated. His mother says he has been feeding well and passing adequate amounts of urine. He appears healthy. He is at the 60th percentile for length and 40th percentile for weight. His temperature is 37.3°C (99.1°F), pulse is 130/min, respirations are 49/min and blood pressure is 62/40 mm Hg. A thrill is present over the third left intercostal space. A 5/6 holosystolic murmur is heard over the left lower sternal border. An echocardiography shows a 3-mm membranous ventricular septal defect. Which of the following is the most appropriate next step in management? (Outpatient Follow-up OR Surgical patch closure)
Low oxygenation in the legs - Pt presents w/ coarctation of aorta 1) Presentation includes: --a) Easy fatigability --b) HTN --c) Lower extremity claudication w/ diminished pulses 2) Murmurs --low-grade systolic ejection murmur Rib nothing - Also presents w/ coarctation of aorta - However, this is a late/chronic symptom of the condition & would present at around age 5 ----(pt is 2 years old)
A 2-year-old boy is brought to the physician by his parents because of difficulty walking and cold feet for the past 2 months. His parents report that he tires quickly from walking. The patient was born at 37 weeks' gestation and has met all developmental milestones. There is no personal or family history of serious illness. He is at the 50th percentile for height and 40th percentile for weight. His temperature is 36.9°C (98.4°F), pulse is 119/min, respirations are 32/min, and blood pressure is 135/85 mm Hg. A grade 2/6 systolic murmur is heard in the left paravertebral region. Pedal pulses are absent. Further evaluation of this patient is most likely to show which of the following findings? (Rib notching OR Low oxygenation in the legs)
Polycythemia - Pt has a VSD - A complication to untreated VSD is Eisenmengers syndrome (shunt reversal) → which leads to cyanosis → the body responds by ↑ EPO, leading to polycythemia Secondary HTN - Secondary HTN is only a complication of Coarctation of aorta ----(pt has VSD)
A 3-year-old boy is brought to the emergency department because of increasing shortness of breath for 2 days. He is at 30th percentile for height and at 25th percentile for weight. His temperature is 37.1°C (98.8°F), pulse is 144/min, respirations are 40/min, and blood pressure is 80/44 mm Hg. Bilateral crackles are heard at the lung bases. A grade 3/6 holosystolic murmur is heard over the left lower sternal border. A grade 2/6 mid-diastolic murmur is heard best in the left fourth intercostal space. Without treatment, this patient is at risk of developing which of the following? (Polycythemia OR Secondary HTN)
Atrialized RV - Pt is taking lithium → lithium is known to cause Ebsteins anomaly → which causes an atrialized RV, tricuspid regurg, & RA enlargement Caudal regression syndrome - Associated w/ maternal GDM
A 30-year-old primigravid woman at 14 weeks' gestation comes to the physician for her first prenatal visit. She reports some nausea and fatigue. She takes lithium for bipolar disorder and completed a course of clindamycin for bacterial vaginosis 12 weeks ago. She works as a teacher at a local school. She smoked a pack of cigarettes daily for 12 years but stopped after finding out that she was pregnant. She does not drink alcohol. Her temperature is 37°C (98.6°F), pulse is 80/min, and blood pressure is 125/80 mm Hg. Pelvic examination shows a uterus consistent in size with a 14-week gestation. There is mild lower extremity edema bilaterally. Urinalysis is within normal limits. The patient's child is at increased risk for developing which of the following complications? (Atrialized RV OR Caudal regression syndrome)
Left Axis deviation on ECG - Pt presents w/ tricuspid atresia 1) Presentation ---Respiratory distress & cyanosis at birth 2) Murmur ---Holosystolic, best heard over left lower sternal border 3) Echo findings ---Defect in the inter-atrial & IV septae ---Imperforate muscular septum btwn the RA & RV ----(which describes a defect in tricuspid valve) 4) Pathophysio ---This leads to an LVH d/t increased pressure & blood flow to the left side of the heart Increased pulmonary vascular marking on CXR - Pulmonary vascular markings are DECREASED in tricuspid atresia b/c there is almost no blood Flow to the right ventricle → leasing to decreased blood flow to the pulmonary system
A 4-day-old male infant is brought to the physician because of respiratory distress and bluish discoloration of his lips and tongue. He was born at term and the antenatal period was uncomplicated. His temperature is 37.3°C (99.1°F), pulse is 170/min, respirations are 65/min, and blood pressure is 70/46 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 82%. A grade 3/6 holosystolic murmur is heard over the left lower sternal border. A single S2 that does not split with respiration is present. Echocardiography shows defects in the interatrial and interventricular septae, as well as an imperforate muscular septum between the right atrium and right ventricle. Further evaluation of this patient is most likely to show which of the following? (Left Axis deviation on ECG OR Increased pulmonary vascular marking on CXR)
PGE1 - Pt presents w/ transposition of great arteries - MGMT includes --1) PGE 1 → b/c we have to keep the PDA open until surgery --2) Surgical correction
A 4-hour-old male newborn has perioral discoloration for the past several minutes. Oxygen by nasal cannula does not improve the cyanosis. He was delivered by cesarean delivery at 37 weeks' gestation to a 38-year-old woman, gravida 3, para 2. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The mother has type 2 diabetes mellitus that was well-controlled during the pregnancy. She has not received any immunizations since her childhood. The newborn's temperature is 37.1°C (98.8°F), pulse is 170/min, respirations are 55/min, and blood pressure is 80/60 mm Hg. Pulse oximetry shows an oxygen saturation of 85%. Cardiopulmonary examination shows a 2/6 holosystolic murmur along the lower left sternal border. The abdomen is soft and non-tender. Echocardiography shows pulmonary arteries arising from the posterior left ventricle, and the aorta rising anteriorly from the right ventricle. Which of the following is the most appropriate next step in the management of this patient? (PGE1 OR Surgery)
Right axis deviation on ECG - Pt presnets w/ TOF 1) Presentation ---"tet" spells → episodes of turning blue that self resolve 2) VSD → which leads to RVH → leading to right axis deviation Pulmonary vascular congestion on CXR - In TOF there is a Right ventricular outflow obstruction to the pulmonary vasculature (b/c of the over-riding aorta) → so there will be NO pulmonary vascular congestion
A 5-month-old boy is brought to the emergency department by his mother because his lips turned blue for several minutes while playing earlier that evening. She reports that he has had similar episodes during feeding that resolved quickly. He was born at term following an uncomplicated pregnancy and delivery. He is at the 25th percentile for length and below the 5th percentile for weight. His temperature is 37°C (98.6°F), pulse is 130/min, blood pressure is 83/55 mm Hg, and respirations are 42/min. Pulse oximetry on room air shows an oxygen saturation of 90%. During the examination, he sits calmly in his mother's lap. He appears well. The patient begins to cry when examination of his throat is attempted; his lips and fingers begin to turn blue. Further evaluation of this patient is most likely to show which of the following? (Right axis deviation on ECG OR Pulmonary vascular congestion on CXR)
Indomethacin infusion - Pt presents w/ a PDA - MGMT of PDA is based on severity of presentation A) Small, asymptomatic PDA ----a) Reassurance & follow-up B) Large, symptomatic PDA ----a) Indomethacin ----(Symptoms include: poor feeding, failure to thrive)
A 5-week-old infant born at 36 weeks' gestation is brought to the physician for a well-child examination. Her mother reports that she previously breastfed her for 15 minutes every 2 hours but now feeds her for 40 minutes every 4 hours. The infant has six wet diapers and two stools daily. She currently weighs 3500 g (7.7 lb) and is 52 cm (20.4 in) in length. Vital signs are with normal limits. Cardiopulmonary examination shows a grade 4/6 continuous murmur heard best at the left infraclavicular area. After confirming the diagnosis via echocardiography, which of the following is the most appropriate next step in management of this patient? (Indomethacin infusion OR Reassurance & follow-up
Decrease in pulmonary vascular resistance - In utero, the pulmonary system is a HIGH pressure system - after birth, the pulmonary system becomes LOW pressure - In pts w/a VSD, a decrease in pulmonary system pressure has led to a DECREASE in RV pressure → leading to increased blood flow from the left ventricle (which has oxygenated blood) to the right ventricle (which has de-oxygenated blood) - B/c more blood from the LV flows into the RV, less of the oxygenated blood flows to the body, leading to cyanosis RVOT obstruction - The murmur described is a VSD - VSD is part of TOF → in which there is RVOT obstruction - However, in TOF there is DECREASED pulmonary vasculature ---(pt has INCREASED pulmonary vasculature)
A previously healthy 2-month-old boy is brought to the physician because of a 10-day history of poor feeding. He used to feed for 20 minutes but now needs 40 minutes. He struggles to breathe and sweats while feeding. He was born at 38 weeks' gestation. He is at the 20th percentile for length and 10th percentile for weight. His vital signs are within normal limits. Pulse oximetry on room air shows an oxygen saturation of 98%. A grade 3/6 holosystolic murmur is heard at the left lower sternal border. An ECG shows left-axis deviation. An x-ray of the chest shows an enlarged left atrium and ventricle and increased pulmonary vascular markings. Doppler echocardiography confirms the presence of an intracardiac shunt. Which of the following is the most likely explanation for the direction of flow of blood across this shunt? (RVOT obstruction OR Decrease in pulmonary vascular resistance)
Passage of catheter thru the nasal cavity not possible - Pt presents w/ choanal atresia 1) Presentation --a) Cyanosis that WORSENS w/ feeding & IMPROVES w/ crying --b) Typically presents as birth 2) MGMT ---a) Diagnostic ------1) INITAL TEST → Catheter thru nasal cavity test ------2) CONFIRMATORY → CT/contrast rhinography Boot-shaped heart on CXR - Diagnostic for tetralogy of fallot - This also presents w/ cyanosis at birth - However, -----1) the cyanosis does NOT worsen w/ feeding or improve w/ crying ----2) also pt would have "tet" spells ------(pt has no Hx of tet spells)
Thirty minutes after delivery, a 3600-g (7-lb 15-oz) newborn has noisy breathing, bluish discoloration of her lips, and intermittent respiratory distress. She was born at 38 weeks' gestation and required bag-mask resuscitation immediately after delivery. Pregnancy was uncomplicated. Her mother has noticed the bluish lip discoloration worsen when she fed and improve when she cried. The patient's pulse is 163/min, respirations are 62/min, and blood pressure is 60/30 mm Hg. The crying infant's lungs are clear to auscultation. Further evaluation of this patient is most likely to show which of the following? (Passage of catheter thru the nasal cavity not possible OR Boot-shaped heart on CXR)
