Successful balloon pulmonary valvuloplasty for congenital valvular pulmonary stenosis in an octogenarian.

While balloon valvuloplasty has been widely used for the treatment of congenital valvular pulmonary stenosis (PS) in children and adults, its use in elderly patients is less common. An 80-year-old woman with congenital valvular PS received valvuloplasty with double-balloon technique. Right ventricle systolic pressure and pulmonary valve systolic pressure gradient decreased from 95 to 44 mm Hg and from 75 to 35 mm Hg, respectively. Follow-up Doppler echocardiography 2 months later showed further decrease in the transvalvular systolic pressure gradient to 29 mm Hg. The patient had symptomatic relief, and no major complication was noted. Balloon pulmonary valvuloplasty can be an effective treatment for elderly patients with congenital valvular PS.     

Prenatal diagnosis of severe tricuspid insufficiency in Ebstein's anomaly with pulmonary atresia and intact ventricular septum: a case report

Pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. A 23-year-old woman was referred at 23 weeks' gestation because of fetal cardiomegaly detected by routine ultrasonography. Ebstein's anomaly with pulmonary atresia was diagnosed by fetal echocardiography. On pulsed Doppler examination, a severe tricuspid regurgitation was detected. Pulmonary atresia was suspected, as forward flow was not detected into pulmonary artery. The fetus was terminated at 25 weeks. At the postmorterm examination Ebstein's anomaly with pulmonary atresia and intact ventricular septum were confirmed. The finding of right atrial enlargement on an early sonogram should prompt the search for tricuspid valve abnormalities with tricuspid regurgitation and the presence or development of pulmonary atresia or stenosis. With the information provided, early detection of tricuspid valve disease should be possible so that the option of therapeutic abortion can be offered.     

球瓣比和球囊长度与儿童经皮球囊肺动脉瓣成形术疗效的关系

目的通过分析不同的球瓣比和球囊长度对儿童经皮球囊肺动脉瓣成形术(PBPV)近期及中远期疗效的影响，探讨最适宜的球瓣比和球囊长度。 方法1987~2005年山东省立医院儿科诊治119例肺动脉瓣狭窄患儿，使用不同球瓣比和长度的扩张球囊行PBPV术，扩张前后测量右室与肺动脉间的峰值压力阶差，并行左侧位右室造影，测量瓣环大小并观察有无右室流出道激惹。术前、术后定期行经胸超声心动图检查，估测最大跨肺动脉瓣压力阶差，并观察肺动脉瓣形态及其反流情况。 结果超大球囊法行PBPV术后，患儿的近期及中远期跨肺动脉瓣压差持续下降，且压差下降率不随球瓣比的增加而增大；术后未发现有肺动脉瓣再狭窄者，所有患儿均有不同程度的肺动脉瓣反流，且反流的程度随时间的延长而加重，并与球瓣比成正相关。对于年龄较小(≤6岁)的儿童，球瓣比大且长度≥40mm的球囊较易引起右室流出道痉挛及三尖瓣反流。中远期三尖瓣反流的发生可能间接继发于肺动脉瓣反流所引起的右室容量负荷过重。 结论PBPV治疗肺动脉瓣狭窄，最佳的球瓣比为1.0~1.2，疗效满意且并发症少；6岁以下儿童宜使用长度<40mm的球囊，可减少右室流出道痉挛及近期三尖瓣反流的发生。     

Amniotic fluid pressure during pregnancy

Intrauterine pressure was measured, at the time of diagnostic amniocentesis or cordocentesis, in 200 pregnancies at 10-38 weeks' gestation. Mean pressure decreased exponentially with gestation from 9 mm Hg at 10 weeks reaching a plateau of 5 mm Hg at 30 weeks. These findings are compatible with Laplace's law of pressure in spheroids.     

经皮球囊肺动脉瓣成形术治疗婴儿和新生儿肺动脉瓣狭窄及闭锁

探讨经皮球囊肺动脉瓣成形（PBPV）术治疗婴儿和新生儿肺动脉瓣狭窄（PS）及室间隔完整型肺动脉瓣闭锁（PA/IVS）的安全性及有效性。方法　2006年1月至2009年4月,广东省心血管病研究所心儿科收治PS及PA/IVS婴儿和新生儿63例。其中危重新生儿20例（31.7％）。术前经超声心动图确诊为PS 56例,PA/IVS 7例。危重新生儿PS需先用直径2.5～4.0 mm小球囊预扩张肺动脉瓣,PA/IVS患儿需先行射频瓣膜打孔术。PBPV术所选球囊直径为肺动脉瓣环径（PVAD）1.2～1.3倍（新生儿所选球囊直径为PVAD 1.0～1.2倍）。结果　全组63例（100％）PBPV术成功。球囊径/瓣环径：1.2±0.1。右室压力明显下降[术前（104.3±32.7） mmHg（1 mmHg = 0.133kPa）,术后（52.0±10.5） mmHg,P < 0.001],跨肺动脉瓣压力阶差明显下降[术前（99.2±23.5） mmHg,术后（27.7±12.4）mmHg,P < 0.001]。平均手术时间（88.1±36.2） min,平均X线曝光时间（16.9±11.1） min。术中6例（9.5％）出现并发症,2例心包积液,2例低氧血症,1例三尖瓣腱索部分撕裂, 1例室上性心动过速。平均随访（19.0±12.3）个月,3例术后因残余中度或重度PS,3个月后再次行PBPV术。本组所有患儿肺动脉瓣仅轻度反流。结论　PBPV术治疗婴儿和新生儿PS及PA/IVS安全、有效。     

Ebstein's anomaly of the tricuspid valve in association with tetralogy of fallot and absent pulmonary valve syndrome

Ebstein's anomaly and absent pulmonary valve syndrome belong to the rarest congenital heart defects. Their association has never been reported so far. We present the unusual case of a fetus at 23 weeks of gestation with Ebstein's anomaly, tetralogy of Fallot, absent pulmonary valve and agenesis of the arterial duct. The main diagnostic features were apical displacement of the septal leaflet of the tricuspid valve with an offset from the mitral valve of 8 mm, a pronounced atrialization of the right ventricle, a large malalignment ventricular septal defect with overriding aorta in combination with absence of the pulmonary valve leaflets, to and fro flow pattern over the stenotic pulmonary valve annulus, turbulent flow in the pulmonary trunk, massive dilatation of the pulmonary trunk plus the pulmonary arteries and a right aortic arch with retroesophageal course of an aberrant left subclavian artery. The arterial duct and the thymus were absent. The remaining fetal anatomy was unremarkable. Amniocentesis revealed a normal male karyotype; 22q11 microdeletion was ruled out. After being counseled on the unfavorable prognosis, the parents opted for termination of pregnancy. The prenatal cardiac findings were confirmed at autopsy; however, a severely hypoplastic thymus was found instead of the suspected aplasia.     

Arterial pressure response to maximal isometric exercise in pregnant women.

We studied the arterial pressure response to maximal leg exercise in 15 healthy volunteers at 29 and 35 weeks' pregnancy and at 8 weeks post partum. Resting mean arterial pressure at 35 weeks' gestation was similar to the postpartum control value of 88 mm Hg (range 73 to 111), but it was reduced by 10% at 29 weeks' gestation. Voluntary maximal effort averaged 2470 N (range 1450 to 3030) in the postpartum period, was similar at 29 weeks, and was reduced by 13% at 35 weeks' gestation. Mean arterial pressure showed a linear increase with force to a median maximum value of 131 (104 to 159) mm Hg, or 49% above the resting value in the postpartum period. The pressure response was unaffected by pregnancy, but was inversely related to maximal force, i.e., the individual's capacity to perform isometric exercise.     

Critical pulmonary stenosis with intact ventricular septum and fetal arrhythmias

A 23-year-old woman, gravida 1, was referred to our hospital for possible fetal distress at 32 weeks of pregnancy. A fetal cardiotochogram showed a reactive pattern, but mild continuous bradycardia and an intermittent pulse were observed, regarded as a sinus type and a type of A-V block, respectively. The continuous deceleration of the heart rate to 95 bpm was observed frequently at 35 weeks, 3 days of gestation. Thus, an emergent cesarean section was performed and a viable 2,082 g female infant was delivered. The neonate gradually became cyanotic, and an echocardiogram was performed. The neonate was regarded as a right ventricular outflow obstruction with intact ventricular septum. Unlike other cases, the infant revealed a moderately developed right ventricle despite a severely stenotic tricuspid valve. The infant died 27 days after birth and an autopsy established the diagnosis of critical pulmonary stenosis with intact ventricular septum. Right ventricular myocardial sinusoidal-coronary artery connections, one of the major features of this type of heart anomaly, was speculated to be involved in the cause of fetal bradyarrhythmias.     

Balloon valvuloplasty for critical aortic stenosis in a low birth weight baby with tracheoesophageal fistula.

The use of valvuloplasty in low birth weight infants with valvular aortic stenosis has not been widely reported. We report a 1556 g 3-day-old female infant with complex heart disease including critical valvular aortic stenosis, bicuspid aortic valve, mitral stenosis, patent ductus arteriosus, and secundum type atrial septal defect. She also had esophageal atresia with a distal tracheoesophageal fistula and trisomy 18. Balloon valvuloplasty was considered to be more dangerous in this patient than in infants with simple critical aortic stenosis without other associated anomalies or low birth weight. Retrograde aortic balloon valvuloplasty was done carefully with the patient intubated and mechanically ventilated. We successfully dilated the stenotic aortic valve, which resulted in a dramatic decrease of the left ventricular pressure from 139/12 mm Hg to 81/11 mm Hg. Despite the high operative risk and poor prognosis, balloon valvuloplasty may be the first feasible step towards the survival of low birth weight infants with valvular aortic stenosis.     

Mitral valve replacement in a twin pregnancy

A 35-year-old female with twin gestation diagnosed with severe mitral stenosis and pulmonary hypertension was successfully treated with open heart surgery for mitral valve replacement (MVR). She gave birth to twins with good Apgar scores at 33 weeks of gestation by cesarean section. Cardiac surgery in singleton pregnancy has been reported extensively. However, there is only a single reported case of MVR following therapeutic abortion of a twin pregnancy in the second trimester. In contrast, we report the first case of mitral valve replacement for severe mitral stenosis and pulmonary hypertension in an ongoing twin pregnancy with successful outcomes.     

Cardiac compliance in fetuses of diabetic women.

OBJECTIVE: To examine possible changes in cardiac function in fetuses of pregestational diabetic mothers. METHODS: We conducted a prospective longitudinal study of 31 women whose pregnancies were between 22 weeks' gestation and term, and who had pregestational diabetes. All diabetic women included in the study had glycosylated hemoglobin lower than 6.5%. All patients included in the study had an early ultrasound confirming gestational age. Doppler studies of the blood flow through the mitral and tricuspid valves were done every 4 weeks using a pulsed-wave Doppler ultrasound device with a 3.5- or 5-MHz transducer. The following indices were calculated from the flow velocity waveforms: the peak velocity during the rapid ventricular filling (E wave) and during the atrial systole (A wave), and the ratio between these velocities (E/A ratio); and the velocity time integral of the atrioventricular blood flow (this integral correlates with volume flow). A comparison between the Doppler indices obtained in fetuses of diabetic women and of normal women was made by using the Mann-Whitney test. RESULTS: Each patient had four to five fetal echocardiographic examinations at 22, 26, 30, 34, and 38 weeks' gestation. The E/A ratio of the mitral and tricuspid valves did not increase in fetuses of diabetic women during the third trimester and was significantly higher in fetuses of nondiabetic women compared with fetuses of diabetic women at 34 and 38 weeks' gestation. The velocity time integral of the mitral and tricuspid valves multiplied by heart rate was higher, but not significantly, in fetuses of nondiabetic women compared with fetuses of diabetic women at 34 and 38 weeks' gestation. The E-wave of the mitral and tricuspid valves increased in both groups throughout gestation. The A-wave of the mitral and tricuspid valves increased only in fetuses of diabetic women throughout the third trimester and was significantly higher at 34 and 38 weeks' gestation compared with fetuses of nondiabetic women. CONCLUSION: Differences in atrioventricular blood flow patterns between fetuses of diabetic women and normal fetuses do not necessarily result from differences in cardiac compliance.     

Hemodynamic and clinical efficacies of catheter balloon percutaneous transvenous mitral commissurotomy: experience of 100 patients with rheumatic mitral stenosis

From January 1987 to December 1988, 100 patients with symptomatic severe rheumatic mitral stenosis underwent percutaneous transvenous mitral commissurotomy (PTMC). The patients included 32 males and 68 females, aged 19-71 years (mean of 41). Mild mitral regurgitation (grade 1 or 2) was present in 23 patients and a history of thromboembolism in 12. One patient had had mitral restenosis after surgical open mitral commissurotomy 9 years earlier. The mitral valve was successfully dilated in 97 patients. PTMC resulted in immediate improvements in hemodynamic measurements. The left atrial pressure decreased from 24.5 +/- 5.3 to 14.8 +/- 5.2 mmHg (p less than 0.001), the mean mitral transvalvular gradient from 13.8 +/- 4.8 to 5.0 +/- 2.8 mmHg (p less than 0.001), and the mean pulmonary artery pressure from 38.8 +/- 12.0 to 30.6 +/- 10.3 mmHg (p less than 0.001). The mitral valve area increased from 1.1 +/- 0.3 to 2.2 +/- 0.8 cm2 (p less than 0.001). The cardiac output increased from 4.5 +/- 1.2 to 4.84 +/- 1.2 L/min (p less than 0.05). The right atrial pressure did not change significantly after PTMC (6.5 +/- 3.8 vs 6.4 +/- 4.0 mmHg). The mitral valve area measured by 2-D echocardiograms increased from 1.04 +/- 0.48 to 1.88 +/- 0.66 cm2 after PTMC (p less than 0.001). All 97 patients were followed for 6-24 months (median of 13) after the PTMC. After an initial recovery period of 1-2 weeks, all patients reported improvements in symptoms and in New York Heart Association (NYHA) functional class by at least one class. A comparison between treadmill exercise test durations before, and 3 months after PTMC, showed an increase from 9.1 +/- 4.3 to 15.4 +/- 3.8 minutes (n = 60; p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)     

Transvaginal fetal echocardiography in early pregnancy: normative data

Fifty-two transvaginal ultrasonographic examinations were performed between 10.0 and 14.9 weeks' gestation for the purpose of documenting normal fetal cardiac anatomy. All standard cardiac projections could be obtained by gestational week 12, and many were imaged by week 11. The four-chamber view was visualized in 90% of fetuses at 12 weeks' gestation and in 100% of fetuses examined at 13 weeks. The aortic root in short axis projection and the left ventricle in long axis view could be imaged in 70% and 40% of fetuses, respectively, by 12 weeks' gestation. Aortic and pulmonary valves were first visualized at 12 weeks, as were five-chamber, ductus arteriosus, and aortic arch views. The mitral and tricuspid valves were resolved in 60% of fetuses by 11 weeks' gestation. This study of normal cardiac anatomy suggests that there may be significant potential for the diagnosis of many fetal cardiac anomalies during the late first and early second trimesters of pregnancy.     

Intrauterine hemodynamics of tricuspid valve dysplasia in a growth-restricted infant

We present a case of fetal tricuspid valve dysplasia, which was diagnosed at 22 weeks of gestation during a routine obstetrical examination. Serial fetal echocardiographic evaluation revealed progressive right ventricular outflow tract obstruction and persistent cardiomegaly. A female infant weighing 1,916 g was delivered by elective cesarean section at 38 weeks of gestation. Longstanding compression of the fetal lungs secondary to the persistent cardiomegaly resulted in severe respiratory distress and cyanosis immediately after birth. Although, mechanical ventilation and continuous infusion of prostaglandin were instituted, the infant died of respiratory failure at 21 days of age. During the pregnancy, the fetus exhibited intrauterine growth restriction, but hydrops did not occur. In regard to the fetal hemodynamics in this cardiac anomaly, transatrial communication is essential for fetal survival. The diameter of the fossa ovalis, which is a marker of transtrial blood flow, was adequate in this case. However, marked enlargement of the right heart associated with regurgitation interfered with left ventricular filling and output, which resulted in restriction of the combined ventricular output and intrauterine fetal growth restriction.     

Left atrial thrombosis in pregnant women with mitral stenosis and sinus rhythm

OBJECTIVE: The purpose of this study was to describe pregnant patients with mitral stenosis who had intracardiac thrombosis in the absence of atrial fibrillation. STUDY DESIGN: We reviewed the clinical course of 3 pregnant women with severe mitral stenosis and normal sinus rhythm who had clinically significant intracardiac thrombosis. RESULTS: The first patient was examined at 21 weeks of gestation with embolic stroke that was the result of left atrial thrombus. A second patient was found to have a large left atrial thrombus that prevented the performance of balloon valvuloplasty. The third patient had left atrial clot that partially occluded the mitral valve orifice and led to the development of pulmonary edema that resulted in an emergent cesarean delivery and anoxic brain injury in the newborn infant. CONCLUSION: Pregnant patients with mitral stenosis in normal sinus rhythm can experience thromboembolic events that can be detrimental to both the mother and the fetus. Anticoagulation therefore should be strongly considered in this group.     

Extensive pulmonary embolism presenting as severe adult respiratory distress syndrome after surgical resection of a cornual pregnancy

A rare case of severe adult respiratory distress syndrome associated with a Po2 of 9 mm Hg after surgical resection of a cornual pregnancy at 10 weeks' gestation was treated successfully with positive end-expiratory pressure. The underlying cause, extensive pulmonary embolism, was subsequently revealed. The patient survived without residual brain damage. The possible causes of adult respiratory distress syndrome in pregnancy are discussed.     

Fetal screening of congenital heart disease

Echocardiograms of two hundred and ninety normal fetuses at 18-39 weeks of gestation were studied with a real-time linear scanner (3.5 MHz). Aortic valvular diameter, pulmonary valvular diameter, mitral valvular diameter, tricuspid valvular diameter and total cardiac dimension were measured. These parameters increased with fetal growth. The ratios of pulmonary to aortic valvular diameter and of tricuspid to mitral valvular diameter were more than 1, suggesting right ventricular dominance in the fetal hearts. The cardio-thoracic ratio was about 50% in this period. The four-chamber view, left ventricular long-axis view, ventricular short-axis view, short-axis view of the great arteries, the view of the aortic arch and the view of the inferior and superior vena cava were also recorded. These six views could be obtained in 9.8% of all subjects at the same time. Detection rates for these views were the greatest in pregnancy at 22-33 (especially 28-29) weeks of gestation. The ventricular short-axis view and short-axis view of the great arteries were easily detected in the dorsoposterior position, whereas the view of the aortic arch was obtained in the dorsoanterior position. It was concluded that fetal echocardiography needed to be performed at least twice, at 22-23 and 28-29 weeks of gestation, and this would be useful in diagnosing some congenital heart diseases.     

Prenatal diagnosis of congenitally corrected transposition of the great arteries. A case report

Congenitally corrected transposition of the great arteries (CTGA) is an uncommon congenital cardiac anomaly. Prenatal sonographic diagnosis of CTGA is very difficult because the ventricular outflow tract may appear to arise correctly from the right and left ventricles. Few cases of CTGA diagnosed in utero have been reported. We report a case of CTGA with complete heart block, ventricular septal defect and pulmonary valve stenosis diagnosed at 30 weeks' gestation after the mother was referred to our hospital because of persistent fetal bradycardia.     

Haemodynamic changes during the puerperium: a Doppler and M-mode echocardiographic study

Summary. Serial haemodynamic investigations were performed in 15 women at 38 weeks gestation and then 2, 6, 12 and 24 weeks after delivery. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic, pulmonary and mitral valves. Cardiac chamber size and ventricular function were investigated by M-mode echocardiography. Flow measurements at the three intracardiac sites correlated closely. Cardiac output fell from a mean of 7.421/min at 38 weeks to 4.961/min at 24 weeks after delivery, a fall of 33%. Most of this decrease (28%) had occurred by 2 weeks. This was associated with a 20% reduction in heart rate and an 18% reduction in stroke volume. By 2 weeks after delivery there was a significant decrease in left atrial dimension and left ventricular end-diastolic dimension. Left ventricular wall thickness and mass declined throughout the period of study as did aortic, pulmonary and mitral valve areas. M-mode derived indices of myocardial contractility were all significantly reduced by 2 weeks and thereafter showed no further change. No haemodynamic differences were found between lactating and non-lactating mothers.     

Rate of prenatal detection of congenital right heart defects

Congenital right heart lesions (including tetralogy of Fallot, pulmonary valve stenosis, pulmonary atresia with intact ventricular septum, Ebstein's anomaly and dysplastic tricuspid valve) account for about 19% of congenital cardiac anomalies. We performed a retrospective study in order to assess the percentage of patients with significant right heart lesions (requiring therapy in the first year of life), which is detected prenatally and referred to a centre for perinatal treatment. From 1/1990 until 12/1997 congenital right heart lesions were diagnosed in 21 fetuses and 190 infants (211 patients. The majority of patients had tetralogy of Fallot (64%), less frequently we found critical pulmonary valve stenosis (9%), pulmonary atresia with intact ventricular septum (9%), tricuspid atresia (14%) and Ebstein's anomaly or dysplastic tricuspid valve (4%). Prenatally the cardiac anomaly was diagnosed in all 21 cases who were referred to our center (10%). The highest referral and detection rate was found among fetuses with Ebstein's anomaly or dysplastic tricuspid valve (5/8 patients = 63%) followed by fetuses with pulmonary atresia and intact ventricular septum (5/20 = 25%), critical pulmonary stenosis (4/18 = 22%) or tricuspid atresia (4/29 = 14%). The prenatal referral rate was disappointing in children with tetralogy of Fallot (3/136 = 2.2%). A higher prenatal detection rate of congenital right heart lesions can be achieved only by an improvement of prenatal screening including the 4-chamber view and the origin of the great arteries. A first step would be the inclusion of the fetal 4-chamber view into the routine examination during the 18th-20th week of pregnancy (stage 1 of a multistage concept of prenatal screening) and by assessment of the outflow tracts and the great arteries in pregnancies associated with risk factors or anomalies of the fetus (stage 2 and 3 of a multistage concept).