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This report describes a rare case of patency of the ductus arteriosus in the setting of ventricular septal defect and rudimentary pulmonary valve. We comment on the fetal, neonatal, and postmortem findings and speculate about the role of the ductus arteriosus in the development of dilatation of the pulmonary arteries in this malformation.
It has been shown in vitro that the lamb ductus arteriosus forms prostaglandins PGE2, PGF2alpha, 6 keto PGF1alpha (and its unstable precursor PGI2). In this study the relative potencies of these endogenous prostaglandins were investigated on isolated lamb ductus arteriosus preparations contracted by exposure to elevated PO2 and indomethacin. All the prostaglandins (except PGF2alpha) relaxed the vessel. This is consistent with the hypothesis that endogenous prostaglandins inhibit the tendency of the vessel to contract in response to oxygen. Only PGE2, however, relaxed the vessel at concentrations below 10(-8)M. PGI2 and 6 keto PGF1alpha had approximately 0.001 and 0.0001 times the activity of PGE2. Although PGE2 has been observed to be a minor product of prostaglandin production in the lamb ductus arteriosus, the tissue's marked sensitivity to PGE2 might make it the most significant prostaglandin in regulating the patency of the vessel.
We report the case of a 47-year-old patient with a patent ductus arteriosus who underwent angiography and percutaneous intravascular ultrasound examination. The case illustrates that intravascular ultrasound contributes to the characterization of patent ductus arteriosus when a surgical or trans-catheter closure is planned, as calcifications of the wall as well as aneurysmal dilatations can be ruled out. Furthermore, measurements of the diameter of the ductus can be made.
OBJECTIVES: This study was performed to define the significance of Doppler color flow mapping in demonstrating reversal of the direction of the normal physiologic flow across the atrial septum and ductus arteriosus in the human fetus. BACKGROUND: Reversal of the physiologic shunting across the ductus arteriosus or atrial septum in utero (i.e., left to right) can be readily identified by Doppler color flow mapping, complemented by pulsed and continuous wave Doppler information. METHODS: We reviewed echocardiograms recorded at our three institutions from 1988 to 1993, which displayed reversal of flow by Doppler color flow in 53 fetuses of gestational age 18 weeks to term. The diagnoses were confirmed by postnatal echocardiography, operation or autopsy. Reversal of shunting was consistently associated with severe heart disease. RESULTS: Reversed atrial shunting was found with severe left heart obstructive lesions, including 19 with hypoplastic left heart syndrome, 3 with critical aortic stenosis, 2 with double-outlet right ventricle and 1 each with an interrupted aortic arch, atrioventricular septal defect and severe left ventricular dysfunction due to dilated cardiomyopathy. Reversed ductus arteriosus shunting was found with severe right heart obstructive lesions, including nine fetuses with pulmonary atresia, six with severe obstructive tricuspid valve abnormalities, five with severe tetralogy of Fallot, four with Ebstein's anomaly and two with single ventricle and pulmonary stenosis. Associated cardiac lesions were common in both groups. Only 3 of the 15 infants who were delivered alive from the reverse ductus arteriosus shunt group and 4 of 12 from the reverse atrial shunt group still survive. CONCLUSIONS: The finding of reversed flow by Doppler color flow mapping during fetal life provides a key to subsequent accurate diagnosis and denotes a spectrum of diseases with a very poor prognosis.
OBJECTIVE: To investigate the echocardiographic characteristics of isolated fetal ductus arteriosus aneurysm (DAA) and the factors influencing its development. METHODS: Fetal echocardiograms for 509 low-risk singleton pregnancies were performed longitudinally from 32 weeks of gestation. The ventricular outflow tracts and great vessels were visualized, focusing on changes in the ductus arteriosus (DA), and fetuses with DAA or ductus arteriosus dilatation (DAD) were identified. Prenatal and perinatal findings were compared between those infants diagnosed postnatally with and those without neonatal DAA. RESULTS: Forty-one of the 509 fetuses (8.1%) studied had neonatal DAA; 2.2% (11/509) had DAA and 5.9% (30/509) DAD before delivery. The mean gestational age at the time of diagnosis of fetal DAA/DAD was 36.9 +/- 1.7 weeks. The mean maximal internal diameter of DAA/DAD was 8.0 +/- 0.8 mm at initial diagnosis, increasing to 10.8 +/- 1.6 mm before delivery. All cases originated from the aortic end of the DA. Compared with fetuses without neonatal DAA, the affected cases had a markedly more curved DA prior to diagnosis, and higher peak velocities at the aortic end of the DA (P < 0.05). Newborns with fetal DAA/DAD had a larger placental weight to birth body weight (BBW) ratio and were less likely to have a BBW appropriate for gestational age (P < 0.05). All cases of fetal DAA/DAD regressed spontaneously. CONCLUSION: Isolated neonatal DAA is a continuity of fetal DAA/DAD. The development of fetal DAA/DAD is likely to be related to the higher peak velocities in the more markedly curved DA occurring in the latter part of the third trimester. Fetal DAA/DAD usually initiates at the aortic end of the DA, followed by progression towards the pulmonary end.
The spontaneous aneurysm of the ductus arteriosus is an extremely rare disease. From a total of 24 adult cases reported in the literature up to 1987, only in one case the ductus was open and other, was recanalized after being closed. We described the second case in the literature in a patient with totally permeable ductus arteriosus who developed a spontaneous aneurysm and endarteritis, showing the great importance of the diagnostic procedures such as echocardiogram, tomography and angiogram, as well as the surgical treatment which was successful in our patient. The probable etiology of this disease is discussed.
During a 23-month period, 25 premature infants underwent ligation of a patent ductus arteriosus performed in the neonatal intensive care unit utilizing a limited posterolateral muscle-retracting incision. This approach afforded adequate exposure with minimal surgical time and trauma. All infants manifested severe respiratory distress and congestive heart failure. Both standard and contrast echocardiographic studies were used for noninvasive preoperative evaluation. Echocardiographic study proved to be a highly reliable and sensitive indicator of ductal patency. Eight infants (32 percent) died at 8 to 225 days of age. The primary cause of death was progressive pulmonary disease with subsequent failure of multiple organ systems. Seventeen (68 percent) of the 25 infants survived to leave the hospital. Advantages of ligation of a patent ductus arteriosus in the neonatal intensive care unit include the elimination of problems of transportation (thermoregulation, ventilation, and loss of lines) and continuity of ongoing care and monitoring. The standard facilities of the neonatal intensive care unit proved completely satisfactory for ligation of a patent ductus arteriosus. Ligation in the neonatal intensive care unit is suggested to minimize potential complications of care in the operating room and transport of these critically ill infants.
Doppler waveforms of the human fetal ductus arteriosus and the branch pulmonary artery are distinct in their shape and might reflect fetal cardiovascular hemodynamics and vessel wall characteristics. The waveform of ductus arteriosus had two peaks, a higher one in systole and a lower one in diastole. Both peaks had slow acceleration and deceleration and looked like two narrow base isosceles triangles. This unique waveform might be due to vessel wall characteristics and an instantaneous pressure gradient between the main pulmonary artery and descending aorta. The waveform of the branch pulmonary artery showed very steep acceleration with the onset of ejection followed by steep decline, then low velocity flow during diastole. The characteristic shape of the branch pulmonary artery might be related to high vascular resistance, decreased capacitance and the earlier reflection wave of pulmonary vessels.
A case of Ehlers-Danlos syndrome in a 3-year-old Chinese girl is described. She had aneurysmal dilatation of patent ductus arteriosus. Resection of the dilated patent ductus arteriosus was performed successfully using cardiopulmonary bypass with total circulatory arrest.
We present clinical, haemodynamic, and angiographic data on a 48-year-old woman with a fistulous aneurysm of the ductus arteriosus. This is the first reported case of a spontaneous fistulous aneurysm of the ductus arteriosus in an adult in which an antemortem diagnosis was made. This lesion typically presents with shortness of breath, hoarseness caused by recurrent laryngeal nerve paralysis, and chest pain, and poses a critical threat to life because of progressive compression, erosion, and haemorrhage into the oesophagus or tracheobronchial tree. Fistulous aneurysm of the ductus arteriosus should be considered in the differential diagnosis of an enlarging mediastinal mass, especially when a continuous heart murmur is noted. Early definitive diagnosis and surgical repair in adults are essential for survival.
In premature neonates a patent ductus arteriosus causes often other complications thus the immediate closure of the patent ductus arteriosus should be preferred or recommended. Performing the surgical closure of the ductus within the incubator can avoid complications during transportation from the premature neonates intensive care unit to the operating room. 25 very small premature neonates or those with another burden in addition were operated upon in the incubator on the premature infants intensive care unit of our hospital during the period from January 1987 to April 1989. No complications occurred during the operation, especially not caused by the strange circumstances for the operating team. Wound infections because of possible bacterial contamination of the incubator or the room were not observed during the postoperative period.
A case of a 49-year-old female with patent ductus arteriosus complicated by severe pulmonary hypertension is presented. The patent ductus arteriosus was successfully closed by the Amplatzer duct occluder. The physical capacity improved from functional NYHA class III at baseline to class I one month after the procedure. A significant reduction of systolic blood pressure in the pulmonary artery and pulmonary resistance was also observed. Indications for transcatheter closure of the patent ductus arteriosus in patients with severe pulmonary hypertension are discussed.
Pulsed Doppler echocardiography has been used to detect continuous turbulent flow in the right pulmonary artery after Blalock-Taussig shunts. Since continuous turbulent flow could also arise from patent ductus arteriosus (frequently present in the neonate), continuous turbulent flow in the right pulmonary artery is not specific for Blalock-Taussig shunt function. We evaluated 35 Blalock-Taussig shunts from suprasternal or high parasternal approach for flow in the right pulmonary artery, and in the Blalock-Taussig shunts themselves. From precordial approach, Doppler evaluations of the main pulmonary artery were also made in search of flow characteristic of patent ductus arteriosus. Doppler detection of flow within the Blalock-Taussig shunts indicated shunt patency, and indicated that continuous turbulent flow in the right pulmonary artery was not due to only patent ductus arteriosus. Shunts were proven patent in 31 patients, occluded in four. Twelve patients also had patent ductus arteriosus. By Doppler, the right pulmonary artery had continuous turbulent flow in 30 of 31 patients in whom the right pulmonary artery was found. From the suprasternal or high parasternal approach, the right Blalock-Taussig shunts were detected by marked continuous turbulent flow directed away from the transducer, between the aortic and superior vena caval flow signals. Left Blalock-Taussig shunts had similar directional continuous turbulent flow. Prosthetic shunts were identified by the dense shunt material. Twenty-four of 31 functional shunts were identified, and all contained continuous turbulent flow. Three left-sided shunts were missed. All four occluded shunts were identified by Doppler, and had no lumen flow within.(ABSTRACT TRUNCATED AT 250 WORDS)
Antenatal closure of the ductus arteriosus is an uncommon event that has been considered as a risk factor for development of congestive heart failure and persistent pulmonary hypertension of the newborn. We report here on a case of antenatal ductus arteriosus closure due to niflumic acid maternal intoxication at 32 weeks of gestation. Fetal extraction was performed few days later because of echographic signs of congestive heart failure. The child survived after 3 days of severe persistent pulmonary hypertension. This case emphasizes the potential risk of niflumic acid during pregnancy and the need of antenatal echographic monitoring for optimal management.
The aim of this study was to determine the effect of varying stretch of the ductus arteriosus on its contractile response to indomethacin and noradrenaline, and its relaxant response to prostaglandin (PG) E2. Isolated rings of ductus arteriosus obtained from fetal New Zealand White rabbits at 28 days gestation were mounted in vitro for measurement of isometric tension by a transducer connected to a vernier control. The responses of the ductus to drugs at varying degrees of passive stretch were corrected to a standard contraction in response to 65 mM potassium at a standard level of stretch. Increasing stretch across the range 1.0-5.9 mN increased the contractile response to indomethacin and noradrenaline (the latter in the presence of 1 microM indomethacin and 1 or 10 nM PGE2) in a virtually identical manner. The sensitivity of the vessel to noradrenaline in the presence and absence of indomethacin and its sensitivity to the relaxant effect of PGE2 in the presence of indomethacin were unaffected by increasing stretch. The extent of contractile tone present which was not inhibited by endogenous PGE2 increased with increasing stretch. It was concluded that: (1) the contractile response to indomethacin can be profoundly altered by the degree of spontaneous tone in the vessel; (2) the magnitude of the contraction induced by indomethacin is not a good index of the degree of inhibition exerted by locally produced PGs in the vessel; and (3) locally produced PGs are likely to have a physiological role in maintaining patency of the ductus arteriosus in utero.
M-mode echocardiographic features suggesting a patent ductus arteriosus are based on two groups of indirect criteria: dilation of the left cardiac cavities and changes of systolic time intervals. The reliability of the first group of criteria has been questioned in fluid-limited, mechanically ventilated preterm infants. The sensitivity of the systolic time intervals in the same circumstances is investigated. Twenty-three patients with a large patent ductus arteriosus were selected. Review of their echocardiograms shows that the sensitivity of the various criteria (expressed as percentage of positivity) was as follows: inversion of the ratio of left ventricular preejection period to right ventricular preejection period, 91.3%; left ventricular preejection period to left ventricular ejection time over right ventricular preejection period to right ventricular ejection time less than 1,83%; left atrium dilation, 74%; shortening of left ventricular preejection period, 70%; dilation of left ventricular internal dimensions in diastole, 65%; increase in left atrium/aorta, 52%; and decrease of left ventricular preejection period to left ventricular ejection time, 48%. Three criteria involving time intervals (left ventricular preejection period to right ventricular preejection period, left ventricular preejection period, and left ventricular preejection period to left ventricular ejection time) had 100% specificity. The lowest specificity was found with criteria involving the left atrium (left atrial to aortic root ratio 75% and left atrium 63%). It is concluded that study of systolic time intervals is a reliable means of detecting preterm infants with hemodynamically significant left-to-right shunt through a patent ductus arteriosus even if the infants are mechanically ventilated and fluid restricted.
We report an adult male cadaver with persistence of the ductus arteriosus and a right retroesophageal artery. The retroesophageal artery was first appreciated as a retropharyngeal mass during the dissection of this area. Both the left and right vertebral arteries originated in normal fashion. The thoracic duct of our specimen drained into the junction of the left internal jugular and left subclavian veins. We believe this to be the first report of simultaneous right retroesophageal subclavian artery and patent ductus arteriosus. The anatomy and embryology of these two anomalies in the same specimen are discussed.
A 59-year-old man with an aneurysm of the ductus arteriosus is described. Macroscopically and histologically it was ascertained that the aneurysm had originated from the ductus arteriosus itself. We suggest the following pathogenesis: after obliteration of the pulmonary end, the aneurysm was formed, and as a result of a gradual increase in pressure in the lumen, the obliterated pulmonary end, the aneurysm was formed, and as a result of a gradual increase in pressure in the lumen, the obliterated pulmonary end recanalized.