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These findings may explain the dramatic decompensation seen in fetuses with uncontrolled tachycardia cheap generic nasonex nasal spray canada allergy symptoms in fall. In the fetus generic 18 gm nasonex nasal spray with mastercard can allergy shots upset your stomach, congestive heart failure can be defined as an inadequate oxygen delivery for normal end-organ function order nasonex nasal spray 18gm free shipping zyprexa allergy symptoms. Therefore order nasonex nasal spray online from canada allergy forecast tucson, regardless of the etiology, indices of diastolic dysfunction tend to precede those of systolic dysfunction. Markers of diastolic dysfunction in the fetus include atrioventricular valvar regurgitation, umbilical venous pulsations, flow reversal in the ductus venosus, or monophasic atrioventricular valve inflow. As heart failure progresses in the fetus, fluid may accumulate in the pericardial space, in the pleural space, in the peritoneal cavity, and in the soft tissues. Fluid accumulation in two or more compartments clinically defines hydrops fetalis, a risk factor for morbidity and mortality in a variety of disease states (119,129,130). Finally, systolic dysfunction is a late and ominous finding in fetal cardiac dysfunction. A shortening fraction of <28% is considered to be abnormal, regardless of gestational age. The cutoff point for predicting poor fetal outcome, however, has varied between studies, and has ranged from <6 to <10. The heart failure score is 10 if there are no abnormal signs and reflects 2 points for each of five categories: hydrops, venous Doppler, heart size, cardiac function, and arterial Doppler. Fetal congestive heart failure: correlation of Tei-Index and Cardiovascular-Score. Usefulness of a new Doppler index for assessing both ventricular functions and pulmonary circulation in newborn piglet with hypoxic pulmonary hypertension. Cardiac output can be estimated by echocardiography from the product of the cross-sectional area of the semilunar valve, the heart rate, and the velocity time integral. Smaller defects may be visualized, but it can be much more challenging to distinguish these from artifact. Although large defects can often easily be visualized by 2D imaging, the diagnosis should always be confirmed by color Doppler imaging, which usually demonstrates bidirectional flow across the defects. False positives are high, as the membranous septum is the thinnest part of the septum, and can appear to be missing even when present. Perimembranous defects may be missed if redundant tricuspid valve tissue covers the defect, preventing visualization of shunting. With normal segmental anatomy, the aortic valve and/or tricuspid valve will always be in the image as a border of the defect. If one starts from a four-chamber view, and sweeps cephalad, the defect is visible just as aortic valve comes into view. There is also a higher association with a right aortic arch which can be detected and may trigger further genetic workup. They are typically detected by color sweeps using a low Nyquist limit in the four-chamber and low short axis views, but may also be noted by 2D imaging without color (Fig. High short axis imaging is the best view to distinguish doubly committed subarterial from perimembranous defects. Studies suggest that between 2% and 31% of muscular defects close during fetal life, with another 19% to 75% closing during the next 12 months (145,147,148,149). For perimembranous defects, studies suggest 4% to 35% of these close in fetal life, and another 1% to 23% in first 12 months, with an estimated 42% needing surgery (146,148,149). Short-axis imaging across the atrioventricular valves also allows for close inspection of the left atrioventricular valve for evidence of a common valve or zone of apposition/cleft (Fig. Color Doppler should always be used, as this may allow better detection of the degree of ventricular level shunting, as this will significantly affect counseling. Color Doppler can also be used to assess the degree of fetal atrioventricular valvar regurgitation, which has been shown to correlate closely with the degree of postnatal regurgitation (150). There is a near common atrium, a large ventricular septal component of the defect, and a common atrioventricular valve. View of the low short axis of the ventricles showing a normal mitral valve without evidence of an atrioventricular septal defect. Of the major congenital heart lesions, it is the least commonly detected in utero (153). Recently, the addition of outflow tract evaluation to routine screening has improved its detection (Fig. Thus careful inspection of the ventricular septum, atrioventricular valves, and outflow tracts is important (Fig. A: Long axis view of the parallel outflow tracts, showing the rightward aorta and leftward pulmonary artery. B: Oblique view showing the pulmonary artery arising from the left ventricle and the aorta arising from the right ventricle. C: Oblique view with color Doppler imaging demonstrating a ventricular septal defect. The link between these defects is further highlighted by the common association with 22q11. Distinguishing these lesions and searching for known associated lesions is of the utmost importance. Anterior and superior deviation of the infundibular septum is a pathognomonic feature (Fig. Determining whether the pulmonary arteries are confluent (image 16B), and the source of pulmonary flow are crucial. Where pulmonary flow is provided by aortopulmonary collateral arteries, flow is typically stable at birth. In the case of pulmonary flow supplied by a ductus arteriosus, the ductus is often small and tortuous. A: Four-chamber view demonstrating the left-handed topology, with right atrium connecting to the right- sided left ventricle, and the left atrium connecting to the left-sided right ventricle. B: View slightly angulated more cephalic from the four-chamber view, showing the posterior pulmonary artery arising from the left ventricle. C: View similar to that in panel B, but now the right pulmonary artery and ductus arteriosus are easily seen. D: Continuing to sweep cephalic, the anterior-superior mildly hypoplastic aorta is seen arising from the hypoplastic right ventricle. A: The infundibular septum is deviated anteriorly, resulting in a narrow right ventricular outflow tract, overriding aorta, and a large ventricular septal defect. Truncus Arteriosus Truncus arteriosus (also known as common arterial trunk) is a conotruncal defect characterized by a single outlet from the heart which gives risk to both the systemic and pulmonary blood flow (Fig. Determining the source of pulmonary blood flow is essential to distinguishing these two lesions. Additionally, the identification of a dysplastic, regurgitant, truncal valve can aid in making this distinction. Identifying aortic arch interruption is crucial, as this would signal the need for prostaglandin administration upon delivery and neonatal surgery to establish stable systemic blood flow. Follow up prenatal echocardiography is important, as progressive truncal regurgitation, fetal hydrops and in utero death may develop (184,185). There is usually a loss of fibrous continuity between the mitral valve and the posterior semilunar valve.
Additional strategies that minimize postoperative hospital stay include routine use of the diuretics including spironolactone nasonex nasal spray 18 gm for sale allergy symptoms guinea pig, an aldosterone antagonist 18gm nasonex nasal spray with visa allergy medicine for cough, and furosemide discount nasonex nasal spray 18gm otc allergy medicine 0027. Supplemental oxygen is used as a pulmonary vasodilator buy nasonex nasal spray 18gm overnight delivery allergy testing yakima wa, and afterload reduction is given to improve cardiac output and lower single-ventricle filling pressures (375). Outcomes for Staged Palliation Most mortality associated with the staged surgical approach occurs during and after stage 1 palliation, with recent cumulative early and interstage mortality in the 5% to 30% range (88,273,376,377). Improved outcome has been associated with early diagnosis, preoperative stabilization, early repair, systematic management approaches, and increased monitoring both in-hospital and at home (86,88,345). Patient-related characteristics are increasingly recognized as risk factors for early and intermediate mortality after stage 1 palliation. Few studies have reported worsened outcomes in patients with prematurity, low birth weight, extracardiac anomalies, genetic syndromes, and/or additional cardiac anomalies. Patients with any of these characteristics have been designated as “high-risk” for staged palliation due to early operative mortality rates of 30% to 50% compared to 10% to 15% operative mortality in patients without the any of the aforementioned characteristics, the “standard-risk” cohort (90,378). We recently reported that intensive perioperative monitoring, early goal-directed treatment of shock and greater resource utilization offset the vulnerability of “high-risk” patients resulting in comparable operative survival in “high-risk” and “standard-risk” patients, 87% versus 95%, respectively. In this series, ability to achieve stage 2 palliation or progression to transplant in lieu of stage 2 palliation was comparable between risk groups. Overall, for this cohort of 162 consecutive patients, operative survival was 91%, 1-year survival was 90%, and survival at last follow-up was 86%. Although cardiac catheterization has been commonly preformed prior to stages 2 and 3 it may be indicated during the neonatal period or as part of post-Fontan management. One-year survival and survival to date are lower in high-risk patients compared to standard-risk patients (p = 0. Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. This was performed in a group of patients that were listed for cardiac transplantation. Cardiac catheterization after stage 1 palliation and prior to stage 2 palliation may be indicated for shunt stenosis, atrial septal defect enlargement or recurrent arch obstruction. Information obtained at catheterization would include the measurement of pulmonary artery pressure, pulmonary capillary wedge pressure, right ventricular systolic and diastolic pressures, and pressures in the ascending and descending aorta. The operators should be prepared to perform interventions as needed on the pulmonary arteries, atrial septum, and arch. In selected patients in whom clinical or anatomic concerns are absent by history, physical examination, and echocardiography, cardiac catheterization may not be necessary prior to stage 2 palliation (381). Indications may include excessive cyanosis that may be due to venovenous collateral or stenotic cavopulmonary connections or branch pulmonary artery stenoses. Catheter intervention for aortic arch narrowing occasionally may also be necessary after stage 2 palliation (249,360). Catheterization is routinely performed prior to the completion Fontan operation in many institutions. Important measurements to determine suitability of Fontan palliation include; pulmonary artery pressure, pulmonary capillary wedge pressure, and ventricular end-diastolic pressure. Cardiac catheterization following the Fontan operation may be necessary if there are anatomic or physiologic concerns not easily elucidated by noninvasive imaging techniques. Some centers routinely perform cardiac catheterization 6 to 12 months after the Fontan procedure with consideration for fenestration closure following hemodynamic assessment. In a report of five patients who underwent the Fontan operation with this technique, all returned home in 24 hours, however several patients required subsequent intervention for baffle leak (245). Late Fontan Concerns Staged palliation for single-ventricle physiology has undergone a series of surgical revisions that have reduced early postoperative Fontan mortality from 20% to less than 2% (391,392). Despite the significant morbidities associated with the Fontan operation, overall late mortality (range 4 months to 18 years) continues to decrease from 25% in the early experience to 5% in the recent era (392,393). Indications for successful Fontan have been modified from the initial “Ten Commandments” described by Choussat and Fontan. This list does specify physiologic risk factors for a failing Fontan that prevail and relate to ventricular performance, atrioventricular and aortic valve function, and pulmonary circulation (395). In addition, more complex anatomy that requires main pulmonary artery to ascending aorta anastomoses or ventricular septal defect enlargement, both indicators of ventricular outflow obstruction, has been identified as a risk factor for late morbidity. Ventricular Dysfunction Volume unloading provided by staged palliation results in reduction in ventricular size and wall thickness that, in turn, increases contractility and ventricular performance. Regardless of the early success with staged palliation, late ventricular dysfunction after the Fontan operation may ensue due to morphologic/structural features of the single right systemic ventricle, residual obstructive lesions, and/or atrioventricular valve insufficiency. The failing systemic ventricle after staged palliation can be attributed to systolic dysfunction, diastolic dysfunction, or both (396,398,399,400). Systolic dysfunction is characterized by reduced contractility and an ejection fraction of less than 50%. Diastolic dysfunction is more difficult to define, but is evident by increased ventricular end-diastolic pressure and the rate of ventricular relaxation (401,402). As a result, late ventricular dysfunction and subsequent failure of Fontan circulation become clinically evident with symptoms of lower functional class, exercise intolerance, dyspnea, fatigue, and syncope (403,404). Hypoxemia Slight hypoxemia with SaO in the low 90s is common after Fontan completion even when residual atrial-level2 shunts (fenestrations) are absent (380,395). This desaturation is thought to result from coronary sinus blood return to the pulmonary venous atrium, and/or ventilation/perfusion imbalances within the lung. Desaturation also commonly occurs in patients with residual anatomic shunts such as a persistent atrial-level shunt (fenestration) or acquired collateral circulation within the lung. Venovenous collaterals which drain directly into the left atrium or pulmonary venous circulation can also serve as a source of arterial desaturation after Fontan palliation. The collateral circulation that forms after Fontan palliation plays no role in gas exchange, produces right-to-left intrapulmonary shunts and might contribute to progressive ventricular dysfunction as a source of chronic volume overload (405). Hence, the impact intrapulmonary collateral circulation on oxygen saturation is variable but is often most pronounced in the presence of progressive ventricular dysfunction. This elevation in abdominal venous pressures presumably leads to intestinal congestion, lymphatic obstruction, and enteric protein loss (409). Diastolic dysfunction, as mentioned previously, that results in low cardiac output in the face of elevated venous pressures, or even with venous pressures considered normal for Fontan physiology (<15 mm Hg), predisposes the patient to mesenteric ischemia and subsequent intestinal mucosal injury leading to the onset of enteric protein losses (395,409). If the above therapies prove unsuccessful, cardiac transplantation can be offered. They suggested that budesonide resulted in an improvement of serum albumin within 6 months and that low-dose therapy must be continued in order to result in a sustained effect. They credit improved survival to a systematic multipronged approach including medical-, surgical-, interventional catheter–based and noncardiac disease management (415). Thromboembolism Patients with Fontan circulation have a life-long risk of thromboembolic complications, particularly stroke and pulmonary embolism. In a large series by Coon, the reported prevalence of thrombus formation as detected by transthoracic echocardiography was 8. In smaller series, the diagnosis of thrombus formation was more common with transesophageal echo with a reported prevalence of 17% to 30% (418). The high rate of thrombus formation is postulated to be predominately secondary to venous stasis and impaired cardiac output that is inherent to single-ventricle circulation.
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Melody transcatheter valve: histopathology and clinical implications of nine explanted devices order nasonex nasal spray line allergy forecast salt lake city. Comparison of the outcome of porcine bioprosthetic versus mechanical prosthetic replacement of the tricuspid valve in the Ebstein anomaly nasonex nasal spray 18gm generic allergy symptoms nausea and dizziness. Thrombotic obstruction of a melody valve-in-valve used for prosthetic tricuspid stenosis 18gm nasonex nasal spray with mastercard allergy forecast napa ca. Valvular and structural heart disease: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition) discount nasonex nasal spray online amex allergy drops cost. The clinical challenge of bridging anticoagulation with low-molecular-weight heparin in patients with mechanical prosthetic heart valves: an evidence-based comparative review focusing on anticoagulation options in pregnant and nonpregnant patients. Maternal complications and pregnancy outcome in women with mechanical prosthetic heart valves treated with enoxaparin. A prospective trial showing the safety of adjusted-dose enoxaparin for thromboprophylaxis of pregnant women with mechanical prosthetic heart valves. Thrombolysis of prosthetic tricuspid valve thrombosis with human recombinant tissue plasminogen activator in an adolescent. Doppler echocardiographic evaluation of streptokinase lysis of thrombosed right-sided St. Thrombolytic therapy for prosthetic valve thrombosis in children: two case reports and review of the literature. Assessing the outcome of systemic tissue plasminogen activator for the management of venous and arterial thrombosis in pediatrics. Intra-atrial tissue plasminogen activator infusion for prosthetic valve thrombosis. Antithrombotic management of patients with prosthetic heart valves: current evidence and future trends. Bleeding and thrombotic emergencies in pediatric cardiac intensive care: unchecked balances. Intracardiac thrombosis diagnosed by echocardiography in childhood: predisposing and etiological factors. Surgical thrombectomy of two left ventricular thrombi in a child with acute myocarditis. Perioperative management of antithrombotic therapy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Antithrombotic and thrombolytic therapy for valvular disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Prosthetic mitral valve thrombosis: can fluoroscopy predict the efficacy of thrombolytic treatment? The role of tissue plasminogen activator in the successful treatment of infected cardiac thrombus in children. Early intracardiac thrombosis in preterm infants and thrombolysis with recombinant tissue type plasminogen activator. Management of preterm infants with intracardiac thrombi: use of thrombolytic agents. Successful thrombolytic therapy for acute massive pulmonary thrombosis after total cavo- pulmonary shunt. Successful treatment of infective endocarditis with recombinant tissue plasminogen activator. Successful thrombolysis of acute left atrial thrombi in two pediatric patients following interventional cardiac catheterization. Management of a large organized intraatrial catheter-tip thrombus in a child with acquired immunodeficiency syndrome using escalating tissue plasminogen activator infusions. Use of tissue plasminogen activator for femoral artery thrombosis following transcatheter coil occlusion of patent ductus arteriosus. Treatment of infective endocarditis with recombinant tissue plasminogen activator. Therapeutic application of intrapericardial tissue plasminogen activator in a 4-month-old child with complex fibropurulent pericarditis. Thrombosis of an extracardiac Fontan tunnel: combined treatment of thrombolysis and stenting. The use of recombinant tissue plasminogen activator in the management of infective intracardiac thrombi in pre-term infants with thrombocytopaenia. Successful treatment of a thrombus in the left aortic coronary sinus in a child with systemic lupus erythematosus. Clinical experience with alteplase in the management of intracardiac and major cardiac vessels thrombosis in pediatrics: a case series. An institutional approach to interventional strategies for complete vascular occlusions. The use of recombinant tissue-type plasminogen activator in a newborn with an intracardiac thrombus developed during extracorporeal membrane oxygenation. Cardiac findings and long-term thromboembolic outcomes following pulmonary embolism in children: a combined retrospective-prospective inception cohort study. Clinical experience with recombinant tissue plasminogen activator in the management of intracardiac and arterial thrombosis in children. Intracardiac thrombus in children: the fine equilibrium between the risk and the benefit. Successful thrombolysis following enoxaparin therapy in two pediatric patients with congenital heart disease presenting with intracardiac and cerebral thrombosis. Additional potential factors supported by general retrospective observational studies or expert opinion include protein-losing enteropathy, prolonged pleural effusions, prolonged immobilization, ventricular dysfunction, arrhythmia, presence of thrombogenic foreign material, atrial-level fenestration, Kawashima connection, and an abnormal thrombophilia profile. Considering the role of the heart as the engine of circulatory propulsion, cardiac structural aberrations may impact capacity for blood flow delivery, with important downstream consequences affecting end-organ functionality. Organs not receiving adequate blood flow with decreased oxygen delivery may malfunction, or develop poorly, leading to long-term complications. There can also be consequences following treatment of heart disease in the developing, immature human infant or child while organs are in a vulnerable, at risk state. Even in cases that are deemed successfully treated by current standards, residual hemodynamic disturbances may persist which can exert either acute, overt changes or indolent, subclinical alterations in end-organ performance. Chromosomal or genetic anomalies influence multiple developing cell lines and often result in abnormalities of both the cardiac and noncardiac systems. The most commonly associated major noncardiac anomalies were musculoskeletal (24%), anomalies of the urinary tract (14%), gastrointestinal system (11%), and central nervous system (11%) (4). Such a child would also be at risk for a multitude of additional noncardiac consequences, including dysmorphology, neurodevelopmental delay, musculoskeletal abnormalities, and a host of other organ system abnormalities. These findings have their origin in the nondisjunction of chromosome 21, resulting in the constellation of findings recognized as Down syndrome. Patterns of phenotypic findings may recur in various patients suggesting an association, although a genetic explanation may not be all that clear.
The relationship between maternal cardiac status and fetal outcomes may be manifested by changes in uterine and umbilical Doppler flow patterns (40) proven 18 gm nasonex nasal spray allergy medicine ears. Hemodynamic and hormonal changes of pregnancy may continue to impact maternal outcomes late after pregnancy (41 order generic nasonex nasal spray online allergy medicine 2013,42 nasonex nasal spray 18gm line allergy forecast santa fe,43 buy nasonex nasal spray on line amex allergy forecast new orleans,44,45). For example, adverse cardiac events late after pregnancy occurred more often in women who had adverse cardiac events during pregnancy (Fig. Pregnancy has been associated with an increased likelihood of requiring valve intervention late after pregnancy in women with moderate or severe aortic stenosis (45). At this time, the full extent and mechanisms of the late effects of pregnancy on the heart are poorly understood. Pregnancy outcomes stratified solely by diagnosis can be helpful, but in addition it is important to consider the specific surgical history, the history of prior cardiac events, the functional status of the woman and ventricular and valve function, since individual variation in these factors may influence risk over and above the risk imparted by diagnosis alone. Potential complications include atrial arrhythmias and heart failure, particularly if the shunt is large. If cardiac shunts are associated with pulmonary hypertension, risk is dominated by the impact of the elevated pulmonary vascular resistance, which is discussed elsewhere in this chapter. Right Ventricular Outflow Tract Obstruction If pulmonic stenosis is mild or has been previously corrected surgically or by valvuloplasty, it is typically well tolerated during pregnancy (32,46,51). In severe pulmonic stenosis, the increase in preload associated with pregnancy may not be tolerated and may result in atrial arrhythmias or right heart failure. Thus, correction of severe pulmonic stenosis prior to pregnancy should be considered. If decompensation develops during pregnancy, balloon valvuloplasty can be carried out if initial medical therapy proves insufficient (52). Although one group has reported high rates of obstetric and fetal complications in women with pulmonary stenosis (53), this differs from experience reported elsewhere (32,46,51). In general pregnancy is well tolerated, but risk of complications is increased in the presence of such residua and surgical sequelae. In one series, maternal complications including symptomatic right heart failure, arrhythmias, or both occurred in 12% of pregnancies (54), though other studies have reported lower adverse event rates (55,56,57). Adverse maternal cardiac events have been reported in association with maternal cardiac factors (left ventricular dysfunction, severe pulmonary hypertension, severe pulmonic regurgitation with right ventricular dysfunction or right ventricular outflow tract obstruction) and obstetric risk factors (twin pregnancies) (55,56). Following biventricular repair for double-outlet right ventricle a low risk for maternal cardiac complications was reported in one series of 19 pregnancies; however, fetal and neonatal risks were increased (58). Left Ventricular Outflow Tract Obstruction Significant left ventricular outflow tract obstruction most commonly occurs as a result of aortic stenosis related to bicuspid aortic valve disease and limits the ability of the heart to increase cardiac output. During pregnancy, all of these factors contribute to an increased propensity to heart failure, ischemia, or hypotension. Bicuspid aortic disease is sometimes associated with ascending aortopathy or coarctation of the aorta, which confer additional risks during pregnancy. However, women with significant aortic stenosis continue to be at risk for heart failure, arrhythmias, and angina (49,51,59,60,61). Women with symptomatic aortic stenosis should undergo surgical correction prior to pregnancy (62). Management of asymptomatic women with severe aortic stenosis is more controversial and careful risk stratification prior to pregnancy is required. In selected women, aortic balloon valvuloplasty may provide short- term palliation prior to a planned pregnancy. In general, prophylactic surgery is not advocated in women with asymptomatic aortic stenosis who otherwise would not be candidates for valve surgery if pregnancy were not a consideration. Palliation by balloon valvuloplasty can be accomplished during pregnancy, if necessary when anatomy allows (63). Pregnancy may increase the risk of cardiac events late after pregnancy; women with aortic stenosis who have been pregnant are more likely to require aortic valve replacement when compared to a matched control group of women who have not been pregnant (45,60). Aortic dissection has been reported in women with bicuspid aortic valve and aortopathy although overall risk is lower than in women with aortopathy associated with Marfan syndrome (64). The approach to the aortopathy associated with bicuspid aortic valve at some centers is to offer empiric beta-blockade and serial echocardiographic assessment during pregnancy. Coarctation of the Aorta In the current era, most women with coarctation of the aorta will have undergone repair prior to pregnancy. Even when there is no residual coarctation, persistent or recurrent systemic hypertension may manifest after repair. Significant coarctation of the aorta impedes delivery of blood to the arterial tree distal to the coarctation site; during pregnancy this may impact on the placental circulation. Upper body hypertension and concomitant aortic valve disease pose additional risks. Maternal mortality has been reported, but this is rare in contemporary series (65,66). Women with repaired coarctation are at increased risk for pregnancy-induced hypertension and preeclampsia (32,46,67). The risk of hypertension is highest in women with unrepaired coarctation in proportion to the degree of residual gradient (65,66). Overtreatment of upper body hypertension during pregnancy could potentially result in hypotension distal to the coarctation site with adverse impact on oxygen delivery to the fetus. Intrauterine growth restriction and premature labor are more common in women with unrepaired coarctation. However, these women are at high risk for cardiac complications during pregnancy (68) such that most experts advise against pregnancy in the presence of 2 aortopathy (aortic size index >2. Some experts have recommended against pregnancy in Turner syndrome even with a normal aorta. Marfan Syndrome In Marfan syndrome, increased cardiac output, hypervolemia, and the hormonal milieu of pregnancy contribute to the increased risk of aortic dilation and dissection. Women with smaller aortic root dimensions are at lower risk for aortic complications than those with a dilated aortic root (>40 mm) or those with prior aortic root surgery (70). Favorable pregnancy outcomes have been reported in women with aortic root size less than 45 mm prior to pregnancy (41,71). On the other hand, a European consensus document estimates that 1% of women with aortic diameters less than 40 mm and no significant aortic or mitral regurgitation will develop dissection or other serious maternal cardiac complication and that 10% of women with an aortic diameter more than 40 mm will develop dissection (72). Unfortunately, prophylactic root replacement prior to pregnancy or the presence of a normal- sized aortic root does not guarantee a safe pregnancy as it is possible for dissection to occur in an aorta that appears “normal” or in the segment distal to a prior repair. Patients with significant aortic root dilation should receive preconception counseling. For women with Marfan syndrome whose aortic size is normal, counseling should incorporate discussion of the increasing risk with increasing maternal age. Women with Marfan syndrome have a 50% chance of transmitting the syndrome to offspring. In addition, they are at increased risk for fetal, neonatal, and obstetric complications, the most common of which is pre-term delivery due to premature rupture of membranes and cervical incompetence. Ebstein Anomaly There is substantial variability in the phenotype of Ebstein anomaly and the ability of the heart to tolerate a pregnancy varies according to the severity of the disease. Women with milder anatomic variations who are acyanotic can expect to have an uncomplicated pregnancy, whereas women with severe Ebstein anomaly may be unable to tolerate the increased preload and cardiac output of pregnancy and as a consequence are at risk for functional deterioration, right heart failure, and arrhythmia (75,76).