An unusual cause of airway obstruction
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《美国医学杂志》
Department of Pediatrics & Pathology, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai-400 022, India
An eight-month-old infant presented to us with fever and cough for three days, and breathlessness and cyanosis of one day's duration. There was a history of recurrent episodes of lower respiratory tract infections, often associated with wheezing since once month of age. The infant had been diagnosed clinically elsewhere to have congenital heart disease and had been advised further investigation and management at a tertiary center. There was no history of cyanosis in the past. On examination, the infant had central cyanosis. There were no dysmorphic features. The heart rate was 160 beats/minute, with all peripheral pulses well-felt. The respiratory rate was 68/minute with severe suprasternal and subcostal retractions, The blood pressure was 76/54 mm of Hg in the right upper limb in the supine position.
Examination of the respiratory system revealed almost absent breath sounds bilaterally. Examination of the cardiovascular system revealed a precordial bulge with the apical impulse being hyperdynamic. The first heart sound was normally heard while the second heart sound was single. A grade 4 pansystolic murmur as well as mid-diastolic murmur were heard in the pulmonary area. Abdominal examination revealeld a liver of 2 cm and a just palpable spleen. Other systems were essentially normal.
Investigations showed hemoglobin of 12g/dl and packed cell volume of 40%. Arterial blood gases showed pH of 7.131, PaCO2 84.4 mm of Hg, PaO2 46.4 mm of Hg, SaO2 69.4%, HCO3 27 meq/L and SBE - 1.33 suggesting severe respiratory acidosis with hypoxia. A chest X-ray showed cardiomegaly with severe hyperinflation of both lung fields Figure1. 2D-echocardiography showed tetralogy of Fallot with absent pulmonary valve syndrome.[3] There was a single mal-aligned ventricular septal defect with bi-directional shunt, a significant right ventricular tract outflow obstruction and moderately severe pulmonary regurgitation. The pulmonary arteries were aneurysmal along with an ostium secundum atrial septal defect with a left-to-right shunt and trivial tricuspid regurgitation.
At admission, the patient was intubated and ventilated but continued to deteriorate with no improvement in the arterial blood gases. Opinion was sought from the cardiothoracic surgeons, for urgent surgery, however, within 4 hours of admission, the patient had a cardiac arrest from which he could not be resuscitated. Post-mortem showed an enlarged globular heart with apex formed by both ventricles. The aorta and pulmonary trunk were parallel to each other, the aorta being situated anteriorly and to the right. The right atrium was dilated with a patent foramen ovale and normal tricuspid valve. The right ventricle was dilated with a 5 mm membranous ventricular septal defect. The pulmonary artery was stenotic with post-stenotic dilatation, compressing on the bifurcation of the trachea. The left atrial cavity was small and the mitral valve was normal. The left ventricle was hypertrophied. The lungs showed wide-spread areas of atelectasis.
Absent pulmonary valve syndrome (APVS) is a rare malformation that is estimated to occur in 3-6% of patients with tetralogy of Fallot and is characterized by rudimentary or nearly absent pulmonary valve tissue with marked dilatation of the pulmonary arteries.[1] Presentation is variable. Patients may be acyanotic or minimally cyanotic. Compression of the airways by the dilated pulmonary arteries may result in significant respiratory compromise.[1] Respiratory problems can very in severity from none to severe respiratory distress requiring mechanical ventilation as in our patient.
Patients with severe bronchial obstruction present a distinct management problem. If an infant develops respiratory acidosis with retention of carbon dioxide, assisted ventilation is indicated, however, weaning from a ventilator may be difficult. Surgical repair techniques vary with the particular anatomy in a given patient, especially the severity of pulmonary dilatation[2]. The best management of patients with severe pulmonic regurgitation, dilated pulmonary arteries and respiratory complications remains uncertain, with mortality for surgical repair in these patients being high. Early outcome has been shown to improve when plication of the dilated pulmonary arteries is performed to relieve airway obstruction.[2] The role of pulmonary valve implantation, the incidence of persistent respiratory symptoms and the need for re-operation are unclear. Many patients may require repeat plication and pulmonary valve implantation.
Acknowledgement
We wish to thank Dr. M.E. Yeolekar, Dean of our institution for permitting the publication of this manuscript.
References
1. Fyler DC. Tetralogy of Fallot. In Nadas' Pediatric Cardiology. Philadelphia; Hanley & Belfus, 1992; 486-488.
2. Godart, F. Houyel L, Lacour-Gayet F et al. Absent pulmonary valve syndrome: Surgical treatment and considerations. Ann Thorac Surg 1996; 62: 136-142.
3. McDonell BE, Raff GW, Gaynor JW et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg 1999; 67(5): 1391-1395.(Juvekar Manisha, Shanbag )
An eight-month-old infant presented to us with fever and cough for three days, and breathlessness and cyanosis of one day's duration. There was a history of recurrent episodes of lower respiratory tract infections, often associated with wheezing since once month of age. The infant had been diagnosed clinically elsewhere to have congenital heart disease and had been advised further investigation and management at a tertiary center. There was no history of cyanosis in the past. On examination, the infant had central cyanosis. There were no dysmorphic features. The heart rate was 160 beats/minute, with all peripheral pulses well-felt. The respiratory rate was 68/minute with severe suprasternal and subcostal retractions, The blood pressure was 76/54 mm of Hg in the right upper limb in the supine position.
Examination of the respiratory system revealed almost absent breath sounds bilaterally. Examination of the cardiovascular system revealed a precordial bulge with the apical impulse being hyperdynamic. The first heart sound was normally heard while the second heart sound was single. A grade 4 pansystolic murmur as well as mid-diastolic murmur were heard in the pulmonary area. Abdominal examination revealeld a liver of 2 cm and a just palpable spleen. Other systems were essentially normal.
Investigations showed hemoglobin of 12g/dl and packed cell volume of 40%. Arterial blood gases showed pH of 7.131, PaCO2 84.4 mm of Hg, PaO2 46.4 mm of Hg, SaO2 69.4%, HCO3 27 meq/L and SBE - 1.33 suggesting severe respiratory acidosis with hypoxia. A chest X-ray showed cardiomegaly with severe hyperinflation of both lung fields Figure1. 2D-echocardiography showed tetralogy of Fallot with absent pulmonary valve syndrome.[3] There was a single mal-aligned ventricular septal defect with bi-directional shunt, a significant right ventricular tract outflow obstruction and moderately severe pulmonary regurgitation. The pulmonary arteries were aneurysmal along with an ostium secundum atrial septal defect with a left-to-right shunt and trivial tricuspid regurgitation.
At admission, the patient was intubated and ventilated but continued to deteriorate with no improvement in the arterial blood gases. Opinion was sought from the cardiothoracic surgeons, for urgent surgery, however, within 4 hours of admission, the patient had a cardiac arrest from which he could not be resuscitated. Post-mortem showed an enlarged globular heart with apex formed by both ventricles. The aorta and pulmonary trunk were parallel to each other, the aorta being situated anteriorly and to the right. The right atrium was dilated with a patent foramen ovale and normal tricuspid valve. The right ventricle was dilated with a 5 mm membranous ventricular septal defect. The pulmonary artery was stenotic with post-stenotic dilatation, compressing on the bifurcation of the trachea. The left atrial cavity was small and the mitral valve was normal. The left ventricle was hypertrophied. The lungs showed wide-spread areas of atelectasis.
Absent pulmonary valve syndrome (APVS) is a rare malformation that is estimated to occur in 3-6% of patients with tetralogy of Fallot and is characterized by rudimentary or nearly absent pulmonary valve tissue with marked dilatation of the pulmonary arteries.[1] Presentation is variable. Patients may be acyanotic or minimally cyanotic. Compression of the airways by the dilated pulmonary arteries may result in significant respiratory compromise.[1] Respiratory problems can very in severity from none to severe respiratory distress requiring mechanical ventilation as in our patient.
Patients with severe bronchial obstruction present a distinct management problem. If an infant develops respiratory acidosis with retention of carbon dioxide, assisted ventilation is indicated, however, weaning from a ventilator may be difficult. Surgical repair techniques vary with the particular anatomy in a given patient, especially the severity of pulmonary dilatation[2]. The best management of patients with severe pulmonic regurgitation, dilated pulmonary arteries and respiratory complications remains uncertain, with mortality for surgical repair in these patients being high. Early outcome has been shown to improve when plication of the dilated pulmonary arteries is performed to relieve airway obstruction.[2] The role of pulmonary valve implantation, the incidence of persistent respiratory symptoms and the need for re-operation are unclear. Many patients may require repeat plication and pulmonary valve implantation.
Acknowledgement
We wish to thank Dr. M.E. Yeolekar, Dean of our institution for permitting the publication of this manuscript.
References
1. Fyler DC. Tetralogy of Fallot. In Nadas' Pediatric Cardiology. Philadelphia; Hanley & Belfus, 1992; 486-488.
2. Godart, F. Houyel L, Lacour-Gayet F et al. Absent pulmonary valve syndrome: Surgical treatment and considerations. Ann Thorac Surg 1996; 62: 136-142.
3. McDonell BE, Raff GW, Gaynor JW et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg 1999; 67(5): 1391-1395.(Juvekar Manisha, Shanbag )