Persistence of the fetal circulation

A 43-year-old woman with bilateral Adie's tonic pupils had a segment of one iris sphincter that contracted spontaneously every few seconds, but was not influenced by light, near vision, or eye movements.     

Persistence of fetal cardiopulmonary circulation: one manifestation of transient tachypnea of the newborn

Five cyanotic newborn infants underwent cardiac catheterization between 8 and 36 hours of age with a tentative diagnosis of cyanotic congenital heart disease. All had normal cardiovascular anatomy. Cyanosis was the result of persistence of fetal cardiopulmonary circulation with right-to-left shunting across the ductus arteriosus. In all infants, cyanosis resolved spontaneously and the infants survived without sequelae. Admission chest roentgenograms of all infants showed marked hyperinflation of the lungs. Except for severe hypoxemia, the clinical presentation, chest films, and course of illness of these infants were consistent with transient tachypnea of the newborn. It is proposed that an increase in pulmonary vascular resistance, due to hyperinflation of the lungs, was the mechanism which reopened the fetal cardiopulmonary circulatory channels and produced hypoxemia, and that these infants suffered from a rare manifestation of a usually benign newborn respiratory condition. Further, given these pathophysiologic mechanisms, the use of continuous transpulmonary pressure gradients in the management of such infants would be contraindicated.     

Acromegalic cardiomyopathy: an echocardiographic study

Thirty eight acromegalic patients (A) and a control group (C) of subjects without heart disease, were studied with echocardiography. Acromegalies were divided in two groups, A1 and A2, who had increase or normal serum growth hormone (GH) levels respectively after treatment (pituitary adenectomy and/or bromocriptine), at the time of the study. In acromegalic patients (A) mean left ventricular (LV) dimensions were normal while LV wall and septal thickness, LV mass and left atrial (LA) dimension were increased compared to control subjects. LVH was present in 79% of acromegalic patients. Asymmetric septal hypertrophy (ASH) was found in 10,5% of our patients. In group A1, IVS, LVPW, LVMM/m2 were significantly increased as compared to group A2. Fractional shortening (FS), ejection fraction (EF), mean velocity of circumferential fibre shortening (Vcf), frequency-normalized Vcf (Vcfn), posterior left ventricular wall velocity (PWV), and normalized PWV (PWVn) were normal in both groups. In patients with active acromegaly (Al) IVS and LVMM/m2 correlated well with the total duration of the disease (r=0.550 p less than 0.01 for IVS; r=0.624 p less than 0.01 for LVMM/m2) and with the duration of acromegaly before treatment (r=0.568, p less than 0.01 for IVS; r=0.500 p less than 0.01 for LVMM/m2). Furthermore a positive correlation was found between IVS and GH levels (r=0,550 p less than 0.01). Concomitant coronary artery disease and or hypertension did not seem to play any role in causing the above mentioned echocardiographic changes. Echocardiography is useful in assessing the cardiac involvement in patients with acromegaly.     

Cardiovascular response to sudden strenuous exercise: an exercise echocardiographic study

We studied the response in left ventricular (LV) internal dimensions and posterior wall thickness during the performance of sudden strenuous exercise without warm-up (SSE) and sudden strenuous exercise with warm-up (SSEw) in 15 healthy, untrained college-aged males (26 +/- 5.0 yr). Measurements of left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVESD), stroke dimension (SD = LVEDD-LVESD), fractional shortening (FS = SD/EDD) and posterior wall thickness (PWT) were obtained from continuous 2-D targeted on M-mode echocardiography. Continuous EKG and blood pressure were obtained at rest and during the final 10 s of SSE (30 s of upright leg cycle ergometry of 400 W at 80 rpm). SSEw was preceded by warm-up exercise (6 min of graded leg cycle exercise of 2-min stages initial load 30 W, increasing in 30-W increments at 60 rpm, followed immediately by SSE). Our findings revealed that there were no significant differences in the LV internal dimensions (LVEDD, LVESD, FS, PWT), HR max, RPP max, ECG, and MAP max between SSE and SSEw. Sudden strenuous exercise without warm-up is not associated with a reduced LV function. The results of this study are contradictory to previous findings that have suggested that SSE is associated with transient global left ventricular (LV) dysfunction.     

Persistent fetal circulation: an evolving clinical and radiographic concept of pulmonary hypertension of the newborn

The roentgenographic presentations of 11 newborn infants with hypoxemia secondary to pulmonary vasospasm and subsequent right-to-left shunting of blood through the foramen ovale and/or ductus arteriosus (persistent fetal circulation) are described (P. F. C. Syndrome). One infant had radiographically normal lungs, while ten had pulmonary parenchymal abnormalities including hyaline membrane disease [4], meconium aspiration syndrome [4], or an ill defined pattern of retained lung fluid [2]. The roentgenographic appearance of the lungs, however, was discordant with the severe hypoxemia observed in most. Heart size was variable but some degree of cardiomegaly was commonly present. Tolazoline, a potent vasodilator, was useful diagnostically and may have resulted in increased survival. An expanded clinical and roentgeonographic concept of the PFC syndrome is suggested.     

Old myocardial infarct: echocardiographic study

A large number of patients survives many years after an acute myocardial infarction (AMI). Echocardiographic study in patients with a very old myocardial infarction (VOMI) can certainly contribute to a better understanding of anatomical and functional damage of the heart. AIM OF THE STUDY: To describe the echocardiographic pattern and to analyze differences between patients with anterior (ant.) and inferior (inf.) VOMI and subjects treated or not with a thrombolytic agent (T+ and T-). METHODS, DESIGN OBSERVATIONAL STUDY: We performed an M-B mode and color-doppler echocardiographic examination of 136 patients (M/F: 130/6), mean age 64.4 +/- 9 years, with an isolated VOMI that is least 5 years old (mean 9.5 +/- 3.1; range 5-16 years), with a good visualization of left ventricular (lv) endocardial profile and without left bundle branch block or valvulopathy is related to myocardial infarction. We established electrocardiographic site and thrombolytic treatment on a documental basis dating from AMI. End diastolic volume index (EDVi), end systolic volume index (ESVi), ejection fraction (EF), wall motion score index (WMSI), left atrial antero-posterior diameter (AD) and presence of any lv aneurysm, scar, thrombus, mitral regurgitation (MR) were assessed. Data were compared with those of 100 normal subjects (controls) with sex, age and physical settlement similar to those of the patients. RESULTS: EDVi appeared much more enlarged in patients than in controls (p < 0.0001). There was a large difference between ant. VOMI and inf. VOMI (p < 0.0001); where as a smaller increase was noticed in T+ versus (vs) T- patients (p = 0.04). In comparison with controls, a smaller difference was observed in inf. VOMI (p = 0.002). ESVi presented a similar behaviour in patients vs controls and ant. vs inf. VOMI (p < 0.0001), but there was no statistical difference between T+ and T- while a large difference was detected between inf. VOMI and controls (p < 0.0001). EF was lower in patients than in controls (p < 0.0001) and in ant. VOMI rather than in inf. VOMI (p < 0.0001); no statistically relevant difference was seen between T+ and T-, while a large difference was observed between inf. VOMI and controls (p < 0.0001). WMSI appeared to be significantly worse in patients vs controls (p < 0.0001), but there was also a great difference between ant. and inf. VOMI (p < 0.0001). T+ had a better index in comparison with T- (p = 0.02). There was also large difference between inf. VOMI and controls (p < 0.0001). AD was larger in patients than in controls (p < 0.0001), but there was no statistical difference between ant. and inf. VOMI and T+ and T-; in comparison with controls, smaller difference was seen in inf. VOMI (p = 0.04). Aneurysm was seen in 16% of patients, more prevalently in ant. (27%) than in inf. VOMI (5%) (p < or = 0.001). Scarring was seen in 45% of patients and, like aneurysms, more in ant. (69%) than in inf. VOMI (22%) (p < or = 0.001). Thrombus was rarely detected (5%) and only in ant. VOMI (12%) with aneurism (p < or = 0.01 vs inf.). MR was seen in a large number of patients (48%) and in 27% of controls (p < or = 0.001). No significant difference was pointed out between ant. and inf. VOMI. Aneurysm, thrombus and MR (21.8 and 55% respectively) were more prevalent in T- than in T+ (9.3 and 48% respectively), with no statistical significance. Scarring prevailed in T+ (48%) rather than in T- (43%), without any statistical difference. CONCLUSIONS: Patients with VOMI show increased lv volumes, decreased EF and persistence of regional wall motion abnormalities. Volumetric and kinetic modifications mainly involve ant. VOMI, but inf. VOMI also presents similar but smaller modifications. Left atrial dimensions also increase in VOMI. Aneurysms, scars, MR occur frequently; while the presence of thrombus is infrequent. Thrombolytic therapy appears to be a long-term protection from anatomical and functional damage. Echocardiography seems to be the ideal tech     

Transition from fetal to extrauterine circulation

In utero, there is increased pulmonary vascular resistance favoring minimal pulmonary blood flow and right-to-left shunting of blood through the ductus arteriosus and foramen ovale. Delivery initiates a series of complex biochemical and structural changes whereby the neonate assumes those processes critical to survival. This article reviews fetal circulation, the characteristics of the fetal cardiopulmonary system, and the transition from fetal to neonatal circulation. It also discusses the consequences of disrupted transition to extrauterine life and the relationship between fetal cardiopulmonary anatomy and congenital heart disease.     

Peripartum cardiomyopathy: a longitudinal echocardiographic study

OBJECTIVE: Our purpose was to determine echocardiographic trends after initial diagnosis of peripartum cardiomyopathy. STUDY DESIGN: Nine women diagnosed with peripartum cardiomyopathy were prospectively recruited for a longitudinal echocardiographic study. Severe myocardial dysfunction was defined as left ventricular end-diastolic dimension > or = 60 mm + fractional shortening < or = 21%, and mild dysfunction was defined as left ventricular end-diastolic dimension < 60 mm + fractional shortening 22% to 24%. Unpaired t tests were used to compare sample means and Fisher's exact test used to compare discrete variables. RESULTS: All women were seen initially for pulmonary edema. Echocardiography showed decreased systolic function in all women. The mean age at diagnosis was 33.0 +/- 6.9 years. All but one woman had a diagnosis of either chronic hypertension (n = 6) or preeclampsia (n = 2). Four women were first seen ante partum and five post partum (range 1 day to 2 months). Repeat echocardiography was performed in all nine women (median 8 months, range 6 weeks to 5 years). There was no correlation between antepartum or postpartum presentation and cardiovascular status on follow-up (p = 0.3). Values for initial left ventricular end-diastolic dimension, severe versus mild dysfunction (68.3 +/- 7.2 mm vs 55.0 +/- 4.2 mm, p = 0.046), follow-up left ventricular end-diastolic dimension, severe versus mild (68.7 +/- 4.1 mm vs 52.0 +/- 5.7 mm, p = 0.002), and follow-up fractional shortening, severe versus mild (14.6% +/- 5.0% vs 28.5% +/- 9.2%, p = 0.02) are significant. Six of the seven women with severe dysfunction had stable disease in follow-up and one is awaiting heart transplant. One of the two women with mild dysfunction had disease resolution and one had stable disease. CONCLUSION: Patients with severe myocardial dysfunction due to peripartum cardiomyopathy are unlikely to regain normal cardiac function on follow-up.     

Measurement of fetal cells in the maternal circulation

There is evidence that the quantitation of fetal-maternal hemorrhage may be useful in reducing some of the RhoGAM failures in preventing Rh sensitization. The Betke-Kleihauer technique is a well-established method that has been used to estimate the amount of transplacental hemorrhage. Recently, a kit (Fetaldex) has become available for use in the detection and quantitation of fetal-maternal hemorrhage. Using control blood smears, we compared the results of the Fetaldex kit with those results obtained by the Betke-Kleihauer technique. The data indicate that the Fetaldex method is as accurate as the Betke-Kleihauer technique in detecting and measuring fetal red cells in the maternal circulation.     

Hemodynamic performance of four mechanical bileaflet prosthetic valves in the mitral position: an echocardiographic study

Plasma methadone concentrations and its main metabolite D,L-2-ethylidiene-1,5-dimethyl-3,5-diphenylpyrrolidine (EDDP) were determined in 93 patients under methadone maintenance treatment to assess their relationship with heroin use and opioid withdrawal symptoms. Neither plasma concentrations of methadone nor EDDP were significantly different when patients that used heroin in last 3 months were compared with those testing negative for this drug (methadone, 355 +/- 217 versus 369 +/- 216 ng/ml, t = 0.29, P = NS; EDDP, 49 +/- 28 versus 54 +/- 40 ng/ml, t = 0.51, P = NS). No correlation between opioid withdrawal scale scores and plasma concentrations of methadone (r = 0.02, P = NS) and EDDP (r = -0.14, P = NS) was found. Therapeutic drug monitoring during methadone maintenance seems to be useful for assessing compliance with treatment but not for predicting heroin use and subjective withdrawal symptoms.     

Definition of inseparably fused ventricular myocardium in thoracopagus: fetal echocardiographic utility and pathologic refinement

Correlative echocardiographic and pathological findings in a thoracopagus with conjoined hearts are reported. One twin had tricuspid atresia with discordant atrioventricular connections and concordant ventriculoarterial connections. The morphologic right ventricle was hypoplastic and there was a large muscular ventricular septal defect. The other twin had hypoplasia of the mitral valve anulus and left ventricle with double-outlet right ventricle and pulmonary valve atresia. The tricuspid valve was severely insufficient in part because of a large orifice and redundant, elongated leaflets with abnormal chordal attachments. The left ventricles of these two twins shared a perforated common "free wall" with at least two large defects allowing mixing of the circulations at that level. Not all anatomic details were established conclusively by fetal echocardiography; however, sufficient diagnostic information was obtained to support a decision not to aggressively resuscitate these twins after elective cesarean delivery at 31 weeks' gestation.     

Doppler echocardiographic assessment of fetal blood flow redistribution during maternal hyperoxygenation

In a retrospective analysis of the standard protocols for fetal echocardiographic examination, 27 fetuses (mean gestational age 29 +/- 5.4 weeks) with ascites were evaluated. Fetal cardiomegaly (increased heart area/chest area ratio), the presence or absence of atrioventricular valve regurgitation, inferior vena cava and ductus venosus Doppler flow velocity and umbilical vein pulsation were evaluated quantitatively in a group of survivors and non-survivors. A statistically significant difference between the two groups was found for the presence of atrioventricular valve regurgitation (p = 0.003), and for cardiomegaly (p = 0.009). There was no statistical difference for the presence of abnormal venous flow and umbilical pulsation (p > 0.05). Abnormal venous Doppler flow velocities in the inferior vena cava and ductus venosus were observed more frequently than umbilical vein pulsation. The mean heart area/chest area ratios in the group of survivors and in the group of non-survivors were 0.34 +/- 0.09 and 0.48 +/- 0.08, respectively (p < 0.001). The mean gestational ages at the time of diagnosis of ascites in the groups of survivors and non-survivors were 33 +/- 3.3 weeks and 28 +/- 5.1 weeks, respectively (p < 0.05); the mean gestational ages at the time of delivery were 35.6 +/- 2.3 weeks and 33.3 +/- 4.9 weeks, respectively (p = 0.33). In terms of different echocardiographic features as well as the gestational age of the unborn patient with ascites, there is apparently no single indicator of a poor prognosis. Our results suggest that the prognosis is usually poor in a fetus with ascites, when cardiomegaly is detected, as well as the presence of tricuspid and mitral valve regurgitation. This is regardless of the type of venous flow and regardless of the etiology of the ascites.     

Uremic pericarditis: a prospective echocardiographic and clinical study

During a 14 month period 59 male patients with chronic renal failure who were candidates for chronic hemodialysis (HD) were evaluated clinically and echocardiographically for pericarditis. All were evaluated prospectively prior to or at the initiation of HD. Definite pericarditis was present in 8, all of whom were severely uremic and required initiation of HD on a semiemergent basis rather than electively (i.e., preselected level of renal function). In 6 of these 8, pericardial effusion responded to dialysis alone, one required pericardiectomy because of hypotension complicating dialysis, and one expired during a right atriogram. Patients dialyzed on an elective basis were all free of pericarditis at the initiation of HD. Pericarditis arising some months after the initiation of HD was a less frequent problem. It is concluded that (a) the incidence of pericarditis in the uremic state is decreased by early initiation of HD before advanced uremic symptoms have developed; (b) pericarditis present at the initiation of HD usually but not always is resolved with the initiation of HD; (c) echocardiography is an important clinical and epidemiological toole to investigate pericarditis in uremic patient populations.     

The fetal alcohol syndrome: symptoms and pathogenesis

The symptoms of the fetal alcohol syndrome and their frequency of appearance are described based on 41 reports in the literature and on own observations. Experimental evidence is presented proving the lack of cytotoxicity, mutagenicity and teratogenicity of alcohol itself and the intensive cytotoxicity, mutagenicity and teratogenicity of acetaldehyde. Responsibility for the fetal alcohol syndrome is ascribed to acetaldehyde at maternal blood concentrations surpassing 35 micrometer and it is suggested that the raised acetaldehyde level is due to an inherited or acquired defect of mitochondrial aldehyde dehydrogenase. Prospective mothers displaying acetaldehyde levels exceeding 30 micrometer after a drink should be advised against bearing a child.     

Three-dimensional echocardiographic evaluation of fetal heart anatomy and function: acquisition, analysis, and display

The purpose of this work was to assess the functional dynamics and anatomy of the cardiac chambers and great vessels in the fetus (18 to 36 weeks) using in utero three-dimensional ultrasonographic imaging. Fifteen patients were studied using conventional two-dimensional sonographic equipment incorporating a position sensor attached to the transducer and a graphics workstation. Sonographic image data were acquired at 30 images per second and required less than 30 seconds per data set. Fetal heart rate and time in the cardiac cycle were determined and used to synchronize image data for reprojection into a volume at the appropriate part of the cardiac cycle. Volume data were analyzed, rendered, and displayed interactively. Three-dimensional sonographic volume data demonstrated fetal cardiac anatomy from multiple orientations and showed the myocardium, valves, ventricles, and atria clearly. The images showed good correlation with currently available embryologic-anatomic-pathologic data. Dynamic and spatial relationships among chambers, valves, and great vessels were readily appreciated. Three-dimensional sonographic imaging of the fetal heart provides both anatomic and functional information regarding the valves, myocardium, great vessels, and chamber dynamics. Interactive three-dimensional cinegraphic display enhances visualization of cardiac anatomy, which can be difficult to appreciate with two-dimensional methods. The methods presented in this work demonstrate the feasibility of three-dimensional fetal echocardiography.     

AAIR versus DDDR pacing in patients with impaired sinus node chronotropy: an echocardiographic and cardiopulmonary study

The aim of this study was to compare AAIR and DDDR pacing at rest and during exercise. We studied 15 patients (10 men, age 65 +/- 6 years) who had been paced for at least 3 months with activity sensor rate modulated dual chamber pacemakers. All had sick sinus syndrome (SSS) with impaired sinus node chronotropy. The patients underwent a resting echocardiographic evaluation of systolic and diastolic LV function at 60 beats/min during AAIR and DDDR pacing with an AV delay, which ensured complete ventricular activation capture. Cardiac output (CO) was also measured during pacing at 100 beats/min in both pacing modes. Subsequently, the oxygen consumption (VO2AT) and VO2AT pulse at the anaerobic threshold were measured during exercise in AAIR mode and in DDDR mode with an AV delay of 120 ms. The indices of diastolic function showed no significant differences between the two pacing modes, except for patients with a stimulus-R interval > 220 ms, for whom the time velocity integral of LV filling and LV inflow time were significantly lower under AAI than under DDD pacing. At 60 beats/min, CO was higher under AAI than under DDD mode only when the stimulus-R interval was below 220 ms. For stimulus-R intervals longer than 220 ms, and also during pacing at 100 beats/min, the CO was higher in DDD mode. The stimulus-R interval decreased in all patients during exercise. The time to anaerobic threshold, VO2AT, and VO2AT pulse showed no significant differences between the two pacing modes. Our results indicate that, at rest, although AAIR pacing does not improve diastolic function in patients with SSS, it maintains a higher CO than does DDDR pacing in cases where the stimulus-R interval is not excessively prolonged. On exertion, the two pacing modes appear to be equally effective, at least in cases where the stimulus-R interval decreases in AAIR mode.