RADIOLOGICAL QUIZ Vivian J. Harris, M.D.
Fig. 1. A cineangiocardiogram frame shows a catheter in the right ventricle, contrast media in a massively dilated pulmonary artery, and the aorta (R. Miller, M.D.). Fig. 2. Frontal portable chest radiograph.shows partial collapse of several lobes and overexpansion of right lung inferiorly.
Fig. 3. Autopsy specimen shows rudimentary valve ring (white arrow), absent pulmonary cusps, and dilated right and left pulmonary arteries (P. Szantos, M.D.).
A full term female infant had retractions and tachypnea shortly after birth. A grade IVIVI systolic and diastolic murmur along the left sternal border and a thrill palpable over the precordium were noted. An electrocardloqram showed a first degree A-V block and right ventricular hypertrophy. Cardiac catherization was performed at 17 hours of age (a single frame from a cineangiogram is shown in Figure 1). She had labored breathing, copious secretions, many cyanotic spells, and retracted continuously. She lived only 35 days, 20 of them on a respirator. Bronchoscopy on the 24th day revealed compression of both main stem bronchi by pulsating pulmonary arteries. A representative chest radiograph (Fig. 2) shows multiple areas of atelectasis which shifted from lobe to lobe, and from lung to lung, almost daily. The heart and the pulmonary arteries were enlarged. On the basis of this information and that contained in the legends, what is your diagnosis?
(Turn to page 204 for the answer)
From the Department of Pediatric Radiology(V.J.H.,Director), Cook CountyChildren's Hospital,Hektoen Institutefor Medical Research,Chicago, Illinois. Accepted for publication in February 1977. emt
184
RADIOLOGICAL QUIZ (continued from p. 184)
The diagnosis of an absent pulmonary valve was established by cardiac catherization, confirmed by bronchoscopy and also at autopsy (Fig. 3). Prominent clinical features of the entity were respiratory distress, diastolic thrills, "to and fro" murmur along the left sternal border, pulmonary insufficiency murmur, and right ventricular hypertrophy on electrocardiogram. Atelectasis alone at this age is not enough to suggest an absent pulmonary valve; atelectasis is commonly due to aspiration, mucus plug, infections, and tracheal tube compression, but rarely due to bronchial stenosis and an aberrant pulmonary artery. Agenesis of the pulmonary valve is uncommon, and usually recognized in infants although cases have been reported in older children and adults. It is generally associated with other congenital cardiac malformations such as tetralogy of Fallot or ventricular septal defect, and has also been described with atrial septal defect, atrioventricular canal, patent ductus arteriosus, Marfan syndrome, tricuspid and mitral atresia, single coronary artery, double outlet right ventricle, and alone, without other cardiac abnormalities (1, 2). Angiocardiography is valuable in identifying the absent valve and the dilated main pulmonary artery and proximal branches, as well as the commonly associated tetralogy of Fallot (4). Respiratory complications, including labor emphysema secondary to the aneurysmal dilatation of the pulmonary arteries, may be the presenting features (2,3). Aneurysmal dilatation is due to increased right ventricular blood flow or intrinsic disease of the pulmonary artery wall. The pulmonary artery dilatation associated with absent valve leaflets may be attributed to muscular weakness at the base of the valve (5), elastic fiber disruption, or absent pulmonary artery media. Increased pulmonary vascular resistance together with high flow from a ventricular septal defect may contribute to aneurysmal dilatation in an infant (2). The pulmonary valve may have completely absent cusps without valve tissue or only buds of fibrous tissue (4). The pulmonary anulus ranges from complete stenosis to marked narrowing.
204
Department of Pediatric Radiology Cook County Children's Hospital Hektoen Institute for Medical Research 1825 West Harrison St. Chicago, Illinois 60612
REFERENCES 1. Osman, MZ, Meng CC, Girdany BR: Congenital absence of the pulmonary valve: report of eight cases with review of the literature. Am J RoentgenoI106:58-69, May 1969 2. Borg SA, Young LW, Roghair GO: Congenital avalvular pulmonary artery and infantile labor emphysema. A diagnostic correlation. Am J RoentgenoI125:412-421, Oct 1975 3. Kurlander, GJ: Bronchial obstruction from aneurysm of the pulmonary artery associated with absence of the pulmonary valve. J Lancet, 85:129-132, Mar 1965 4. Pernot C, Hoeffel JC, Henry M, et al: Radiological patterns of congenital absence of the pulmonary valve in infants. Radiology 102:619-622, Mar 1972 5. Miller, RA, Lev M, Paul MH: Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation 26:266-78, Aug 1962
Absent Pulmonary Valve Radiological Quiz
Vivian J. Harris, M.D. The author reports a case of an absent pulmonary valve in an infant. Radiographic findings of mild to severe cardiomegaly, with large pulmonary artery branches and evanescent atelectasis, suggested the diagnosis. An angiocardiogram confirmed the greatly enlarged main pulmonary arteries, enlarged right ventricle, and early filling of the aorta via a ventricular septal defect. Dilated pulmonary arteries had compressed adjacent bronchi, with resultant obstructive emphysema or atelectasis. INDEX TERMS: Infants, newborn, cardiovascular system • Infants, newborn, respiratory tract. Pulmonary arteries, abnormalities. (Pulmonary valve, atresia, 5[33].171)
Radiology 125:184, 204, October 1977
Fig. 1. A cineangiocardiogram frame shows a catheter in the right ventricle, contrast media in a massively dilated pulmonary artery, and the aorta (R. Miller, M.D.). Fig. 2. Frontal portable chest radiograph.shows partial collapse of several lobes and overexpansion of right lung inferiorly.
Fig. 3. Autopsy specimen shows rudimentary valve ring (white arrow), absent pulmonary cusps, and dilated right and left pulmonary arteries (P. Szantos, M.D.).
A full term female infant had retractions and tachypnea shortly after birth. A grade IVIVI systolic and diastolic murmur along the left sternal border and a thrill palpable over the precordium were noted. An electrocardloqram showed a first degree A-V block and right ventricular hypertrophy. Cardiac catherization was performed at 17 hours of age (a single frame from a cineangiogram is shown in Figure 1). She had labored breathing, copious secretions, many cyanotic spells, and retracted continuously. She lived only 35 days, 20 of them on a respirator. Bronchoscopy on the 24th day revealed compression of both main stem bronchi by pulsating pulmonary arteries. A representative chest radiograph (Fig. 2) shows multiple areas of atelectasis which shifted from lobe to lobe, and from lung to lung, almost daily. The heart and the pulmonary arteries were enlarged. On the basis of this information and that contained in the legends, what is your diagnosis?
(Turn to page 204 for the answer)
From the Department of Pediatric Radiology(V.J.H.,Director), Cook CountyChildren's Hospital,Hektoen Institutefor Medical Research,Chicago, Illinois. Accepted for publication in February 1977. emt
184
RADIOLOGICAL QUIZ (continued from p. 184)
The diagnosis of an absent pulmonary valve was established by cardiac catherization, confirmed by bronchoscopy and also at autopsy (Fig. 3). Prominent clinical features of the entity were respiratory distress, diastolic thrills, "to and fro" murmur along the left sternal border, pulmonary insufficiency murmur, and right ventricular hypertrophy on electrocardiogram. Atelectasis alone at this age is not enough to suggest an absent pulmonary valve; atelectasis is commonly due to aspiration, mucus plug, infections, and tracheal tube compression, but rarely due to bronchial stenosis and an aberrant pulmonary artery. Agenesis of the pulmonary valve is uncommon, and usually recognized in infants although cases have been reported in older children and adults. It is generally associated with other congenital cardiac malformations such as tetralogy of Fallot or ventricular septal defect, and has also been described with atrial septal defect, atrioventricular canal, patent ductus arteriosus, Marfan syndrome, tricuspid and mitral atresia, single coronary artery, double outlet right ventricle, and alone, without other cardiac abnormalities (1, 2). Angiocardiography is valuable in identifying the absent valve and the dilated main pulmonary artery and proximal branches, as well as the commonly associated tetralogy of Fallot (4). Respiratory complications, including labor emphysema secondary to the aneurysmal dilatation of the pulmonary arteries, may be the presenting features (2,3). Aneurysmal dilatation is due to increased right ventricular blood flow or intrinsic disease of the pulmonary artery wall. The pulmonary artery dilatation associated with absent valve leaflets may be attributed to muscular weakness at the base of the valve (5), elastic fiber disruption, or absent pulmonary artery media. Increased pulmonary vascular resistance together with high flow from a ventricular septal defect may contribute to aneurysmal dilatation in an infant (2). The pulmonary valve may have completely absent cusps without valve tissue or only buds of fibrous tissue (4). The pulmonary anulus ranges from complete stenosis to marked narrowing.
204
Department of Pediatric Radiology Cook County Children's Hospital Hektoen Institute for Medical Research 1825 West Harrison St. Chicago, Illinois 60612
REFERENCES 1. Osman, MZ, Meng CC, Girdany BR: Congenital absence of the pulmonary valve: report of eight cases with review of the literature. Am J RoentgenoI106:58-69, May 1969 2. Borg SA, Young LW, Roghair GO: Congenital avalvular pulmonary artery and infantile labor emphysema. A diagnostic correlation. Am J RoentgenoI125:412-421, Oct 1975 3. Kurlander, GJ: Bronchial obstruction from aneurysm of the pulmonary artery associated with absence of the pulmonary valve. J Lancet, 85:129-132, Mar 1965 4. Pernot C, Hoeffel JC, Henry M, et al: Radiological patterns of congenital absence of the pulmonary valve in infants. Radiology 102:619-622, Mar 1972 5. Miller, RA, Lev M, Paul MH: Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation 26:266-78, Aug 1962
Absent Pulmonary Valve Radiological Quiz
Vivian J. Harris, M.D. The author reports a case of an absent pulmonary valve in an infant. Radiographic findings of mild to severe cardiomegaly, with large pulmonary artery branches and evanescent atelectasis, suggested the diagnosis. An angiocardiogram confirmed the greatly enlarged main pulmonary arteries, enlarged right ventricle, and early filling of the aorta via a ventricular septal defect. Dilated pulmonary arteries had compressed adjacent bronchi, with resultant obstructive emphysema or atelectasis. INDEX TERMS: Infants, newborn, cardiovascular system • Infants, newborn, respiratory tract. Pulmonary arteries, abnormalities. (Pulmonary valve, atresia, 5[33].171)
Radiology 125:184, 204, October 1977
