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Recognition of functional pulmonary atresia by color doppler echocardiography
Recognition of Functional Pulmonary Atresia by Color Doppler Echocardiography Cheng-Liang Lee, MD, PhD, Kai-Sheng Hsieh, MD, Ta-Cheng Huang, Chu-Chung Lin, MD, and Choun-Seng Choong, MD unctional pulmonary atresia in the neonate may represent a F combination of weakened right ventricle from severe tricuspid regurgitation and normal high perinatal pulmonary vascular resistance. By definition, these patients have a normal pulmonary valve and infundibulum. When the perinatal pulmonary resistance falls to the normal range, functional pulmonary atresia may disappear. This situation must be distinguished from anatomic pulmonary atresia with intact ventricular septum to avoid an unnecessary, highrisk operation. To prove functional pulmonary atresia, it is necessary to confirm that the pulmonary valve is normal and can be passed through by a catheter, or the pulmonary artery can be opacified by contrast medium passing from the right ventricle in the catheterization laboratory. But this involves some risk, and may be difficult owing to severe tricuspid regurgitation. Some investigators have described how to distinguish functional pulmonary atresia from anatomic pulmonary atresia.1–3 They all emphasized that functional pulmonary atresia can be diagnosed when there was moderate or severe pulmonary regurgitation, because that phenomenon is incompatible with anatomic pulmonary atresia.
vere Ebstein’s anomaly with enlarged right ventricle and right atrium. There was no anterograde flow from the right ventricle across the pulmonary valve to the pulmo-
MD,
nary artery (Figure 1). Two-dimensional echocardiography revealed that the pulmonary valve was normal but the leaflets did not open. There was a patent ductus arterio-
FIGURE 1. Transthoracic echocardiographic short-axis view. There was no anterograde flow from the right ventricle across the pulmonary valve to the pulmonary artery. Red anterograde flow from the ductus was rebounded (blue retrograde flow) from the pulmonary valve area into the pulmonary artery. There was no pulmonary regurgitation flow across the pulmonary valve.
•••
Recently, we had a newborn with severe Ebstein’s anormaly, a 3-kg newborn referred to our hospital for treatment of cyanotic congenital heart disease. Color Doppler echocardiography revealed seFrom the Department of Pediatrics, Veterans General Hospital-Kaohsiung, Kaohsiung; and the Department of Pediatrics, Jen Ai General Hospital, Pingtung, Taiwan. Dr. Hsieh’s address is: Department of Pediatrics, 386 TaChung 1st Rd, Kaohsiung, Taiwan. E-mail: [email protected]. Manuscript received August 28, 1998; revised manuscript received and accepted October 23, 1998. ©1999 by Excerpta Medica, Inc. All rights reserved.
FIGURE 2. Transthoracic echocardiographic short-axis view. Blue anterograde flow passed through the pulmonary valve into the pulmonary artery with a strong cry. 0002-9149/99/$–see front matter PII S0002-9149(98)01045-5
987
sus flow jet from the aorta to the pulmonary artery with maximal velocity of 2 m/s. When the patent ductus arteriosus flow jet touched the pulmonary valve, the flow jet rebounded back to the pulmonary artery and its branches (Figure 1). There was faint flow passing through the pulmonary valve into the right ventricular outflow tract occasionally. This faint pulmonary regurgitation suggested the diagnosis of functional pulmonary atresia, but was thought inconclusive. When we were performing echocardiography, the newborn suddenly cried intensely, the pulmonary valve opened and right ventricle anterograde flow passed through the pulmonary valve into the pulmonary artery (Figure 2), rendering the diagnosis of functional pulmonary atresia certain. Follow-up echocardiography after 4 days demonstrated that the pulmonary valve could open and the
right ventricle anterograde flow could pass through the pulmonary valve into the pulmonary artery because of decreasing pulmonary vascular resistance. Unfortunately, the newborn died 6 days later due to sepsis. •••
Crying aids right ventricular ejection into the pulmonary artery. This crying phenomenon has not been described previously. This phenomenon may be explained by decreased intrathoracic pressure and pulmonary artery pressure alternating with vigorous inspiration, and decreased intrathoracic pressure resulting in increased right ventricular filling and ejection fractions. When a patient has pulmonary atresia and pulmonary regurgitation is obvious, then functional pulmonary atresia can be diagnosed. When a patient has pulmonary atresia and pulmonary regur-
988 THE AMERICAN JOURNAL OF CARDIOLOGYT
VOL. 83
MARCH 15, 1999
gitation is not obvious, we recommend that physicians stimulate the newborn to cry intensely. If the pulmonary valve opens and right ventricle anterograde flow can pass through the pulmonary valve, then functional pulmonary atresia can be diagnosed. In summary, vigorous crying aids right ventricular ejection into the pulmonary artery. This phenomenon can differentiate functional pulmonary atresia from anatomic pulmonary atresia. 1. Freedom RM, Culham G, Moes F, Olley PM,
Rowe RD. Differentiation of functional and structural pulmonary atresia: role of aortography. Am J Cardiol 1978;41:914-920. 2. Smallhorn JF, Izukawa T, Benson L, Freedom RM. Noninvasive recognition of functional pulmonary atresia by echocardiography. Am J Cardiol 1984;54:925-926. 3. Weinhaus L, Jureidini S, Nouri S, Connors RH. Functional pulmonary atresia: color flow recognition and treatment with extracorporeal membrane oxygenation. Am Heart J 1990;119:980-982.
vere Ebstein’s anomaly with enlarged right ventricle and right atrium. There was no anterograde flow from the right ventricle across the pulmonary valve to the pulmo-
MD,
nary artery (Figure 1). Two-dimensional echocardiography revealed that the pulmonary valve was normal but the leaflets did not open. There was a patent ductus arterio-
FIGURE 1. Transthoracic echocardiographic short-axis view. There was no anterograde flow from the right ventricle across the pulmonary valve to the pulmonary artery. Red anterograde flow from the ductus was rebounded (blue retrograde flow) from the pulmonary valve area into the pulmonary artery. There was no pulmonary regurgitation flow across the pulmonary valve.
•••
Recently, we had a newborn with severe Ebstein’s anormaly, a 3-kg newborn referred to our hospital for treatment of cyanotic congenital heart disease. Color Doppler echocardiography revealed seFrom the Department of Pediatrics, Veterans General Hospital-Kaohsiung, Kaohsiung; and the Department of Pediatrics, Jen Ai General Hospital, Pingtung, Taiwan. Dr. Hsieh’s address is: Department of Pediatrics, 386 TaChung 1st Rd, Kaohsiung, Taiwan. E-mail: [email protected]. Manuscript received August 28, 1998; revised manuscript received and accepted October 23, 1998. ©1999 by Excerpta Medica, Inc. All rights reserved.
FIGURE 2. Transthoracic echocardiographic short-axis view. Blue anterograde flow passed through the pulmonary valve into the pulmonary artery with a strong cry. 0002-9149/99/$–see front matter PII S0002-9149(98)01045-5
987
sus flow jet from the aorta to the pulmonary artery with maximal velocity of 2 m/s. When the patent ductus arteriosus flow jet touched the pulmonary valve, the flow jet rebounded back to the pulmonary artery and its branches (Figure 1). There was faint flow passing through the pulmonary valve into the right ventricular outflow tract occasionally. This faint pulmonary regurgitation suggested the diagnosis of functional pulmonary atresia, but was thought inconclusive. When we were performing echocardiography, the newborn suddenly cried intensely, the pulmonary valve opened and right ventricle anterograde flow passed through the pulmonary valve into the pulmonary artery (Figure 2), rendering the diagnosis of functional pulmonary atresia certain. Follow-up echocardiography after 4 days demonstrated that the pulmonary valve could open and the
right ventricle anterograde flow could pass through the pulmonary valve into the pulmonary artery because of decreasing pulmonary vascular resistance. Unfortunately, the newborn died 6 days later due to sepsis. •••
Crying aids right ventricular ejection into the pulmonary artery. This crying phenomenon has not been described previously. This phenomenon may be explained by decreased intrathoracic pressure and pulmonary artery pressure alternating with vigorous inspiration, and decreased intrathoracic pressure resulting in increased right ventricular filling and ejection fractions. When a patient has pulmonary atresia and pulmonary regurgitation is obvious, then functional pulmonary atresia can be diagnosed. When a patient has pulmonary atresia and pulmonary regur-
988 THE AMERICAN JOURNAL OF CARDIOLOGYT
VOL. 83
MARCH 15, 1999
gitation is not obvious, we recommend that physicians stimulate the newborn to cry intensely. If the pulmonary valve opens and right ventricle anterograde flow can pass through the pulmonary valve, then functional pulmonary atresia can be diagnosed. In summary, vigorous crying aids right ventricular ejection into the pulmonary artery. This phenomenon can differentiate functional pulmonary atresia from anatomic pulmonary atresia. 1. Freedom RM, Culham G, Moes F, Olley PM,
Rowe RD. Differentiation of functional and structural pulmonary atresia: role of aortography. Am J Cardiol 1978;41:914-920. 2. Smallhorn JF, Izukawa T, Benson L, Freedom RM. Noninvasive recognition of functional pulmonary atresia by echocardiography. Am J Cardiol 1984;54:925-926. 3. Weinhaus L, Jureidini S, Nouri S, Connors RH. Functional pulmonary atresia: color flow recognition and treatment with extracorporeal membrane oxygenation. Am Heart J 1990;119:980-982.