Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients.

BACKGROUND: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. METHODS AND RESULTS: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. CONCLUSIONS: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

Sequential magnetic resonance monitoring of pulmonary flow with endovascular stents placed across the pulmonary valve in growing Swine.

BACKGROUND: Patients with endovascular stent implantation for the treatment of right ventricular outflow tract obstruction are often left with incomplete relief of the obstruction and significant pulmonary regurgitation. A noninvasive and reproducible method for monitoring such patients is desirable. MRI in the presence of a stent, however, has to overcome the problem of potential metallic artifacts. METHODS AND RESULTS: Under x-ray fluoroscopic guidance, endovascular nitinol stents were placed across the pulmonary valve in 6 young pigs to induce pulmonary regurgitation. Five additional pigs served as controls. Initial MRI was performed after 2 days (13.5+/-1.8 kg) and follow-up after 3 months (32+/-2.9 kg). Pulmonary flow volumes and regurgitant fraction were quantified by velocity-encoded cine (VEC) MRI through (VEC-TS) and distal to (VEC-DS) the stent. VEC-TS was compared with VEC-DS and volumetric measurements of left and right ventricular stroke volumes provided by cine MRI ("gold standard"). Antegrade and retrograde pulmonary flow volumes by VEC-TS were slightly but significantly less than those with VEC-DS and cine MRI. Excellent correlations (r>0.97) for phasic pulmonary flow volumes as measured by VEC-TS and VEC-DS were shown. Pulmonary regurgitant fraction increased from 32.8+/-15% to 49.6+/-17% (P<0.05) over the course of 3 months with VEC-TS. CONCLUSIONS: MRI demonstrates the progression of pulmonary regurgitation in growing swine. VEC MRI has the ability to quantify pulmonary blood flow inside the lumen of nitinol stents. MRI appears to be ideally suited for monitoring patients with endovascular nitinol stents in the pulmonary artery or pulmonary valve position.

Evaluation of Gianturco coils for closure of large (> or = 3.5 mm) patent ductus arteriosus.

OBJECTIVES: This report evaluates the use of Gianturco coils to close large patent ductus arteriosus (PDAs) (> or = 3.5 mm) and describes transvenous delivery of 0.052-in. (0.132-cm) Gianturco coils. BACKGROUND: Coil closure of PDAs has become increasingly popular. However, the technique has significant limitations when used to close large PDAs. This report evaluates patient characteristics, PDA anatomy, hemodynamic variables, delivery technique and coil geometry to determine predictors of success. METHODS: Between January 1995 and January 1997, 16 of 118 patients undergoing catheterization for PDA closure were found to have large PDAs. Their median age and weight were 14 months (range 3 months to 43 years) and 8.5 kg (range 3.5 to 73), respectively. The mean PDA diameter was 4.3 mm (range 3.5 to 5.9). Closure of PDAs was attempted using transcatheter delivery of 0.038-in. (0.096-cm) and 0.052-in. coils. Differences in clinical, anatomic, hemodynamic and technical variables between successes and failures were compared. RESULTS: Eleven (69%) of 16 patients had successful closure of their PDA. Failures occurred only in patients < 8 months of age with an indexed PDA diameter > 7 mm/m and a pulmonary/systemic flow ratio > or = 2.8:1. Use of 0.052-in. coils tended to reduce the incidence of embolization and the number of coils needed for closure. CONCLUSIONS: Patients > 8 months of age can have successful closure of large PDAs with currently available Gianturco coils. The 0.052-in. Gianturco coils can be used safely to close large PDAs in infants as small as 6 kg. Increased experience and improved coil design may improve closure rates of large PDAs in infants.

Nonlinearity of the left ventricular end-systolic wall stress-velocity of fiber shortening relation in young pigs: a potential pitfall in its use as a single-beat index of contractility.

OBJECTIVES: We sought to evaluate in the young heart the primary assumptions on which the current use of the mean "velocity of fiber shortening corrected for heart rate" as a noninvasive index of contractility are based. BACKGROUND: End-systolic wall stress-velocity of fiber shortening relation has been applied as a single-beat, load-independent index of contractility in children. This use is based on poorly validated assumptions of linearity, parallel shifts with changing contractile state and inotropic sensitivity of the end-systolic wall stress-velocity of fiber shortening relation. METHODS: In eight anesthetized young piglets, 5F mciromanometric catheters were placed in the ascending aorta and balloon occlusion catheters in the descending aorta. End-systolic wall stress and velocity of fiber shortening were calculated from aortic pressure and M-mode echocardiography under six conditions: in three contractile states 1) baseline, 2) increased contractility during dobutamine infusion (10 micrograms/kg per min), and 3) decreased contractility after propranolol injection (1 mg/kg), each at two afterload states (normal and increased load by partial aortic occlusion). RESULTS: Dobutamine increased and propranolol decreased afterload-matched velocity of fiber shortening corrected for heart rate significantly to 140% and 77% of baseline, respectively. However, the slope of end-systolic wall stress-velocity of fiber shortening relation was much greater (251% of baseline) during dobutamine infusion, which also significantly decreased wall stress, and was much less (27% of baseline) after propranolol injection, which increased wall stress. CONCLUSIONS: The velocity of fiber shortening corrected for heart rate did change predictably with changes in contractility and as such can be used noninvasively in the temporal evaluation of individual patients undergoing therapeutic interventions or to define the natural history of a disease process. However, the relation on which it is based is not defined by parallel straight lines across contractile states, so that abnormal single point measurements may reflect only the nonlinearity of the relation rather than abnormalities in contractility. Thus, we recommend that the end-systolic wall stress-velocity of fiber shortening relation should not be used as a single-beat index of contractility.

Interaction between afterload and contractility in the newborn heart: evidence of homeometric autoregulation in the intact circulation.

OBJECTIVES: We undertook the present study to determine whether afterload and contractility interact in the hearts of newborn lambs. We specifically investigated whether stepwise increases in afterload increase contractility. BACKGROUND: Several studies in the isolated and intact adult dog heart have shown that afterload and contractility are not independent determinants of cardiac performance; rather, they interact. Afterload and contractility are unlikely to interact in the newborn heart because the factors that may mediate the interaction in the adult are missing in the newborn. METHODS: We measured contractility at different steady state levels of afterload in seven newborn lambs under complete anesthesia. Contractility was measured by three different indexes: end-systolic pressure-volume relations (slope and volume position); preload-corrected first derivative of left ventricular pressure (dP/dtmax); and preload-corrected stroke work. Left ventricular pressure and volume were measured with a micromanometer and conductance catheter, respectively. Preload and afterload were manipulated by inflating or deflating a balloon catheter in the inferior vena cava and descending thoracic aorta, respectively. Data are expressed as mean value +/- 1 SD. RESULTS: Stepwise increases in afterload increased contractility, independent of which of the three indexes was used. The slope of the end-systolic pressure-volume relation increased from a mean baseline value of 4.44 +/- 2.43 to 6.69 +/- 2.89 kPa/ml at the highest level of afterload. Concomitantly, volume at 14 kPa of the end-systolic pressure-volume relation decreased from 3.34 +/- 1.52 ml at baseline to 1.12 +/- 0.83 ml at the highest afterload. The other two indexes showed qualitatively similar changes. Beats selected from unloading interventions on the basis of the same end-diastolic volume for each level of afterload showed no difference in stroke volume. CONCLUSIONS: This study in newborn lambs demonstrates that stepwise increases in afterload increase contractility considerably and that this enables the heart to maintain stroke volume at different levels of afterload. This forms direct evidence for the existence of homeometric autoregulation in the intact newborn heart.

Complications of pediatric cardiac catheterization: a 3-year study.

To determine the current risk of pediatric cardiac catheterization, the complications and incidents of all catheterizations performed in a pediatric laboratory between January 1986 and October 1988 were prospectively recorded and compared with results from a 1974 study from the same institution. In the current study 1,037 catheterizations, 885 diagnostic and 152 diagnostic/interventional procedures, were performed in 888 patients (aged 1 day to 27 years, median 15.6 months). There were 15 major complications (1.4%), 70 minor complications (6.8%) and 30 incidents (2.9%). Two patients died as a result of the procedure and two as a result of pericatheterization clinical deterioration caused by the cardiac abnormality. The great majority of complications were successfully treated or were self-limited and the patients had no residua. Of patients with 13 nonfatal major complications and 70 minor complications, residua were evident in 7 patients and 3 without evident residua had the potential for sequelae (0.7% and 0.3% of catheterizations). A comparison of the diagnostic and balloon atrial septostomy cases in the present study with similar cases in the 1974 study shows that the incidence of major complications has decreased from 2.9% to 0.9% (p less than 0.0001); minor complications and incidents have decreased from 11.7% to 7.9% (p less than 0.006) and pericatheterization deaths not attributable to catheterization have decreased from 2.8% to 0.2% (p less than 0.0001). Changes in pericatheterization medical management, patient selection for catheterization and catheterization techniques probably account for these improvements.

Metabolic abnormalities in the idiopathic Fanconi syndrome: studies of carbohydrate metabolism in two patients.

Two patients with idiopathic Fanconi syndrome and glucose intolerance were studied from a metabolic perspective. They had fasting hyperglycemia, massive glucosuria, insulinopenia, ketosis, and elevated serum free fatty acids. There was a markedly blunted insulin secretory response to glucagon, tolbutamide, glucose, and arginine. One patient had the findings of diabetic retinopathy and a sensory neuropathy. Neither patient could convert galactose to glucose, but they did not have galactosemia. As a result of these studies, and previous reports in which similar changes were noted, we conclude that diabetes mellitus may occur in patients who have had idiopathic Fanconi syndrome for many years.

Cardiac catheterization of low birth weight infants.

The increased survival of low birth weight infants means that more of these infants may be candidates for catheter interventions. There are few data on the results of cardiac catheterization in this group. This study aimed to analyze, retrospectively, cardiac catheterization of infants weighing < or =2.5 kg, with emphasis on the results of interventions. The complication rates of interventional and diagnostic procedures were compared. One hundred eleven catheterizations were performed in 107 patients between 1985 and 1998. Thirty-one procedures were interventional. Balloon atrial septostomy (n = 16), balloon pulmonary valvuloplasty (n = 10), balloon pulmonary angioplasty (n = 1), and coil occlusion of collateral vessels (n = 3) were all performed successfully. One infant (3%) died while undergoing myocardial biopsy. The reintervention rate for isolated pulmonary valve stenosis was 25% (2 of 8) at 1 month, 57% (4 of 7) at 6 months, and 71% (5 of 7) at 1 year. Complications were significantly more frequent during interventional (13 of 31, 42%) than during diagnostic (13 of 80, 16%) procedures. The most common complications during interventions were arrhythmias (3 of 31, 10%) and respiratory deterioration (3 of 31, 10%). Cardiac catheterization was technically feasible in all patients. Balloon pulmonary valvuloplasty and atrial septostomy provided good palliation in this patient group. The mortality of interventional procedures was low. The high incidence of respiratory complications suggests that low birth weight infants should undergo elective ventilation for interventional cardiac catheterization.

Comparison of M-mode echocardiographic measurement of right ventricular wall thickness obtained by the subcostal and parasternal approach in children.

Right ventricular (RV) wall thickness was measured from M-mode echocardiograms at end-diastole from both the parasternal and subcostal approaches in 50 children of various body surface areas (0.24 to 1.68 m2). The measurements were obtained from M-mode recordings generated from sector scans to ensure precise location and position. Twenty-three children had normal hearts, and 27 had various congenital heart defects that may be associated with RV hypertrophy. Corresponding measurements of the RV wall thickness at end-diastole from the 2 approaches were similar. Subcostal echocardiographic measurement of RV wall thickness was found to be a reliable alternative to parasternal measurement in children with normal hearts and in those with congenital heart disease and RV hypertrophy.

Two-dimensional echocardiographic determination of aortic and pulmonary artery sizes from infancy to adulthood in normal subjects.

The aorta, right pulmonary artery and pulmonary trunk were measured from the 2-dimensional echocardiogram (2-D echo) of 110 normal subjects aged 1 day to 18 years. The vessel diameters were measured from the parasternal short-axis view, the suprasternal long-axis view and the suprasternal short-axis view. Measurements were made at end-systole and at end-diastole and in both an axial and lateral direction where possible. When analyzed with respect to body surface area (BSA), the echocardiographic measurements were linearly related to the square root of the BSA, and there was inequality of variance around the relation. To establish a range of normal values for each vessel dimension, a weighted regression analysis was used to produce estimates of the regression line and a set of tolerance intervals. The systolic vessel dimension was larger than the diastolic vessel dimension and the measurement of a vessel in an axial direction was larger than the measurement of the same vessel in a lateral direction. In general, when a vessel was measured in several views, the largest diameter was obtained using the view that imaged the vessel in cross section. These data on normal values for the echocardiographic measurement of the aorta and pulmonary arteries at different BSAs should be useful for identifying patients with abnormalities in arterial size and for the serial assessment of arterial size in children who have undergone surgical or medical therapy.

Aortoventriculoplasty with the pulmonary autograft: the "Ross-Konno" procedure.

BACKGROUND: For patients with complex left ventricular outflow tract obstruction, including hypoplastic aortic anulus with or without severe diffuse subaortic stenosis, various aortoventriculoplasty procedures (e.g., Konno procedure and its modifications; extended aortic allograft root replacement) are important management options. In younger patients, however, reoperation for valve replacement is inevitably required, and anticoagulation issues pose additional problems. The pulmonary autograft provides a promising option for aortic valve replacement as part of the aortoventriculoplasty procedure in children. Long-term follow up shows that the pulmonary autograft functions well as the systemic arterial (neoaortic) valve and that valve growth occurs. METHODS: Between July 1993 and May 1995, 11 patients 4 days to 17 years old (median 12 months) underwent aortoventriculoplasty with pulmonary autograft (Ross-Konno procedure). The diagnoses were aortic stenosis with or without subaortic stenosis (n = 8), Shone complex (n = 2), and interrupted aortic arch with subaortic stenosis (n = 1). On average, 1.9 previous interventions had been performed per patient, including a previous Konno procedure in one patient. The aortic root was replaced with a pulmonary autograft valve. The left ventricular outflow tract was enlarged with a Dacron polyester fabric patch in two patients, with an allograft aortic patch in two patients and a right ventricular infundibular free wall muscular extension harvested in continuity with the autograft in seven patients. RESULTS: Intraoperative transesophageal echocardiographic assessment revealed mild aortic insufficiency in one patient. One patient had a residual left ventricular outflow tract gradient of 15 mm Hg. Significant complications were cardiac tamponade from bleeding (n = 1) and complete heart block necessitating a permanent pacemaker (n = 1). Follow-up ranged from 2 weeks to 16 months. To date, there have been no late deaths or reoperations. Follow-up echocardiography revealed mild autograft insufficiency in one patient and a 16 mm Hg residual left ventricular outflow tract gradient in one patient. CONCLUSIONS: Initial experience suggests that aortoventriculoplasty with the pulmonary autograft is an excellent alternative for young patients with complex left ventricular outflow tract obstruction. Because the pulmonary autograft has been shown to grow after implantation, reoperation on the left ventricular outflow tract is likely to be avoided.

One-stage complete unifocalization in infants: when should the ventricular septal defect be closed?

BACKGROUND: The decision whether to close the ventricular septal defect at the time of unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals may be difficult. The purpose of this study was to develop morphologic and physiologic methods to aid in deciding whether to close the ventricular septal defect in patients undergoing one-stage unifocalization. METHODS: Between July 1992 and April 1996, 27 infants with pulmonary atresia, ventricular septal defect, and aortopulmonary collaterals were treated at our institution. Midline complete unifocalization was performed in 25 patients-the ventricular septal defect was closed in 17 and left open in eight. Two patients with severe distal collateral stenoses underwent staged unifocalization. Pulmonary artery and collateral sizes were measured from preoperative angiograms and used to calculate the indexed cross-sectional area of the total neopulmonary artery bed. An intraoperative pulmonary flow study previously validated with experiments in neonatal lambs was performed in six patients: the unifocalized neopulmonary arteries were perfused with a known flow and pulmonary artery pressures were recorded. RESULTS: The neopulmonary artery index was greater in patients who underwent ventricular septal defect closure than in those who did not (p = 0.001), although the values did overlap. This index correlated with the postoperative right ventricular/left ventricular pressure ratio (p = 0.037). Mean pulmonary artery pressures obtained during the intraoperative flow study and after bypass were comparable. CONCLUSION: The total neopulmonary artery index correlates with postrepair right ventricular/left ventricular pressure ratio and is useful in deciding when to close the ventricular septal defect if it is larger than 200 mm2/m2. The pulmonary flow study is helpful in deciding whether to close the ventricular septal defect in all patients.

Acute elevation of coronary venous pressure does not affect left ventricular contractility in the normal and stressed swine heart: implications for the Fontan operation.

OBJECTIVE: After the Fontan operation the right atrium and, thus, the coronary sinus are connected to the pulmonary arterial system, which causes the coronary venous pressure to increase. We investigated the acute effects of elevation of coronary venous pressure on baseline hemodynamics, coronary venous flow, and left ventricular contractility. METHODS: In acutely instrumented pigs, during complete right heart bypass and during constant cardiac output, pressure in the right atrium, right ventricle, and coronary sinus was altered by a height-adjustable reservoir. At various levels of coronary venous pressure (up to 4 kPa or up to 30 mm Hg), flow from the reservoir was measured and left ventricular hemodynamics and contractility were measured from catheter-derived left ventricular pressure and (conductance) volume data. Contractility of the left ventricle was assessed by the end-systolic pressure-volume relationship derived during an unloading intervention by adjusting the bypass pump speed. RESULTS: Left ventricular end-diastolic pressure increased slightly (about 5%) with each kilopascal increase in coronary venous pressure, most likely related to diastolic ventricular interaction. No other changes in hemodynamic parameters occurred. Neither coronary venous flow nor left ventricular contractility was influenced by changes in coronary venous pressure. Imposing myocardial stress with dobutamine, 10 microg/kg per minute, did not change these findings. CONCLUSION: Increasing coronary venous pressure to 4 kPa in the intact circulation with intact autoregulation does not affect coronary flow or left ventricular contractility. We found no experimental evidence for the usefulness of diversion of the coronary sinus to the left atrium during Fontan-type operations

Results of 102 cases of complete repair of congenital heart defects in patients weighing 700 to 2500 grams.

BACKGROUND: Published data suggest that low birth weight is a risk factor for poor outcome in corrective surgery for many cardiac defects. Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative operations, with definitive repair delayed. The morbidity associated with such approaches is high. METHODS: Since 1990 complete repair of congenital heart defects (other than patent ductus arteriosus) has been performed in 102 infants no larger than 2500 g (median 2100 g, range 700-2500 g), including 16 no larger than 1500 g. Defects included ventricular septal defect (n = 22), tetralogy of Fallot complexes (n = 20), transposition complexes (n = 13), aortic coarctation (n = 12), interrupted arch (n = 10), truncus arteriosus (n = 8), atrioventricular septal defect (n = 6), total anomalous pulmonary venous return (n = 5), and other (n = 6). RESULTS: Preoperative morbidity was more common among patients referred late for surgical correction. There were 10 early deaths (10%) attributable to cardiac failure (n = 4), arrhythmia (n = 1), multiorgan failure (n = 1), sepsis (n = 1), idiopathic coronary artery intimal necrosis (n = 1), foot gangrene (n = 1), and pulmonary hemorrhage (n = 1). No patient had postbypass intracerebral hemorrhage. At follow-up (median 36 months) there were 8 late deaths, and 8 patients underwent 10 reinterventions. There was no evidence of neurologic sequelae attributable to the operation. CONCLUSIONS: In general, delaying repair of congenital heart defects in low birth weight infants does not confer a benefit and is associated with higher preoperative morbidity. Complete repair of both simple and complex lesions can be achieved in such cases with good results. Growth after repair approximates the normal curve for low birth weight infants without heart disease. It is recommended that such infants, especially when they have symptoms, undergo early surgical repair rather than prolonged medical management or other forms of palliation.

Pediatric angiocardiography with iohexol.

A nonionic contrast medium (iohexol) was evaluated for safety and efficacy in pediatric angiocardiography in this study of 15 patients, age 6 to 82 months. Patients carried a preliminary diagnosis of congenital heart disease. Subjects were injected with iohexol, 350 mg iodine/ml of solution with an average volume of 2.46 ml/kg of body weight at a rate of 9 to 14.5 ml/sec. The parameters evaluated included vital signs, intravascular BP, ECG changes, discomfort, and adverse reactions. No adverse reactions were noted in 14 of 15 patients. No significant changes in axillary temperature of ECG were observed. Intravascular blood pressure showed only moderate changes. After 24 hours, creatine phosphokinase (CPK) plasma concentrations increased significantly. Serum electrolytes remained unchanged. Image quality was deemed excellent considering variations in injection site and flow condition of the heart. Iohexol caused remarkably little discomfort and no adverse effects.

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect.

BACKGROUND: The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD). METHODS: We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998. RESULTS: CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized. CONCLUSIONS: The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.

Intramyocardial hematoma causing cardiac tamponade after repair of Ebstein malformation: erroneous echocardiographic diagnosis as intracavitary thrombus.

Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the right atrium to the atrialized right ventricle. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention.

Circulatory adjustments to postnatal life.

Dramatic changes occur in the cardiovascular system at birth. The circulation changes from one characterized by the presence of central shunts, a relatively low combined ventricular output, right ventricular dominance, and pulmonary vasoconstriction, to a circulation in series with a high cardiac output equally divided between the two ventricles, and a greatly dilated pulmonary vascular bed. To understand the mechanisms that initiate these profound changes, studies that separate the components of the birth process in the chronically instrumented fetus must be continued, along with biochemical studies of isolated tissues to determine the cellular and subcellular events that mediate these changes. Understanding the many processes that control perinatal cardiovascular development will assist the physician in treating those infants in whom the transition from the fetal to the neonatal circulation is abnormal.

Cerebral blood flow velocity: the influence of myocardial contractility on the velocity waveform of brain supplying arteries.

Indices of Doppler-derived velocity waveforms of arteries perfusing the brain are used as relative measures of neonatal brain blood flow. Using a dog model, we investigated the influence of changes in myocardial contractility, induced by dobutamine, on the blood flow velocity waveform of the vertebral artery. The following indices of the velocity waveform were investigated during control states and during 5 or 10 micrograms/kg/min dobutamine infusion: peak systolic flow velocity (PSFV), temporal mean flow velocity (TMFV), end-diastolic flow velocity (EDFV) and acceleration time (ACC-time). PSFV and ACC-time of the vertebral artery showed a strong relationship with myocardial contractile state. These results indicate that PSFV of an artery supplying the brain or indices which combine PSFV with MFV or EDFV should not be used for noninvasive assessment of brain blood flow or cerebral vascular resistance. ACC-time may prove to be very useful in assessing changes in myocardial contractile state.

Fetal chemoreception: a developing story.

The central and peripheral chemoreceptors are critical to the efficient uptake and delivery of oxygen and the removal of carbon dioxide after birth. However, the importance and activity of fetal chemoreception has been questioned, since oxygen uptake and carbon dioxide removal are not regulated in the lungs in the fetus. Early studies suggested that chemotransduction-the conversion of a chemical stimulus to cardiovascular and ventilatory responses via the integration of chemoreceptor stimulation, neural afferent activity and neurohormonal effector mechanisms-was immature in its individual components or their interaction. However, it now appears that the chemoreceptor cascade is structurally and functionally intact in the late-term fetus, and responds actively to normal and other chemical stimuli. The differences between fetal and postnatal chemotransduction appear to be primarily dependent on the central inhibition of the ventilatory response, the inhibitory area being localized to the lateral pons. It appears to be mediated in part by a placental factor which is removed at birth, allowing for the expression of the ventilatory response. The suppression of this response is also responsible for the difference in the heart rate response: the postnatal tachycardia is caused by the lung inflation reflex; when abolished, bradycardia is seen, just as in the fetus. Despite the suppression of the ventilatory component of chemoreception, the fetal carotid chemoreceptor is more important than the aortic, even though it has been considered to be more important to ventilatory than to cardiovascular stability. This review discusses current knowledge of the various components of the mature chemoreceptor cascade, and presents the fetal story within that framework.