Heart Disease in Pregnancy

Heart Disease in Pregnancy
Dr. Mohamed El Sherbiny MD Ob.& Gyn. Senior Consultant Damietta, Egypt

Classification of Heart Diseases in Pregnancy:
Incidence: 1% of all pregnancies
1-Rheumatic valve diseases
(still the most common in the developing countries.)
MS, MR, DM, AS, AR.
2-Congenital disorders
(The most common in developed countries.)
ASD, VSD, PDA, coarcitation of the aorta, Fallot's tetralogy, Eisenminger syndrome and Marfan syndrome.
3-Others: Arrhythmias, ischemic heart disease and cardiomyopathy.

Effect of Pregnancy on Heart Disease
1.Decompensation (heart failure):
• During pregnancy: Precipitated by increased cardiac output especially with anemia (at 28-32 weeks).
• During labor: Due to bearing down and the effort and stress of labor ↑ venous return.
• After delivery: Due to sudden ↑ in venous return (10-20%) with exhausted cardiac reserve or massive emoblization.

Effect of Pregnancy on Heart Disease
2-Rheumatic activity which may cause further damage to the valves.
3-Bacterial endocarditis due to infection by streptococcus viridans in the valves and myocardium.
4-Pregnancy induced low vascular resistance may improve the symptoms of MR, AR & MV prolapse.
So a small family, while young, with 2-3 years spacing is advised before progression of the lesions.

Effect of Heart Disease on Pregnancy
• Intrauterine growth restriction
• Pre-term labor
• Intrauterine fetal death
• Abortion
• Fetal polycythemia.
• Perinatal mortality up to 20%
• Increase fetal congenital HD (from 0.6% to 4.5%) if the mother has CHD.

Prognosis
It depends on:
• The functional capacity of the heart
• Existing complications that increase the cardiac load such as stress, infection and high effort
• Anemia
• Quality of medical care

Diagnosis

Diagnostic Definition of Heart Disease
• 1-Etioloical Diagnosis
• 2-Anatomical Diagnosis
• 3-Functional Classification
• 4-Therapuetic Status
(e.g. patient under digitalis has less prognosis than those of the same functional class without treatment)

Diagnosis of Heart Disease
The physiological adaptations of normal pregnancy can induce symptoms and signs that may be confused with that of heart disease.
Symptoms:
• Dyspnea : ↑HR, consciousness of breathing (progesterone)
• Orthopnea: Supine Hypocaval S.
• Palpitation
• Easily fatigability: Hormonal E&P

Diagnosis of Heart Disease
Signs
• Peripheral edema
• Extrasystole
• SVT
• Prominent pulsation of the neck veins.
• Accentuated 1st H. sound: DD MS
• Systolic murmur: DD Aortic or P. stenosis
• Mammary Souffle: DD P. ductus arteriosis

Clinical Indicators of Heart Disease during Pregnancy
Symptoms:
• Progressive dyspnea or orthopnea
• Nocturnal cough
• Hemoptysis
• Syncope
• Chest pain

Clinical Indicators of Heart Disease during Pregnancy
Signs
Cyanosis
Clubbing of fingers
Persistent neck vein distension
Systolic murmur grade≥ 3/6
Diastolic murmur
Cardiomegaly
Persistent arrhythmia
Pregnant women who have none of these findings rarely have serious heart disease.

Diagnostic Study & Normal Pregnancy Findings
ECG: 15° left-axis deviation
Echocardiography: Tricuspid regurgitation and significantly increased left-atria size and left-ventricular outflow cross sectional area
Chest X’ray: heart silhouette is larger in pregnancy; however, gross cardiomegaly can be excluded.
If indicated, heart catheterization.

Classification of Heart Diseases in Pregnancy
Incidence: 1% of all pregnancies
1-Rheumatic valve diseases
(still the most common in the developing countries)
MS, MR, DM, AS, AR
2-Congenital disorders
(The most common in the developed countries)
ASD,VSD,PDA, coarcitation of the aorta, Fallot's tetralogy , Eisenminger syndrome and Marfan syndrome
3-Others: Arrhythmias,ischemic heart disease and cardiomyopathy.

Preconceptional Counseling

Preconception Care
1-Prenatal folic acid 400-500ug/d
2-Rubella immune status; immunize if not immune.
3-Medications contraindicated in pregnancy should be stopped:
• Warfarin: (Except with mechanical valves ) Embryopathy (abnormal cartilage and bone formation)
• ACE Inhibitors (at all trimesters: Fetal renal dysgenesis)

Preconception Care
4-Managing or correction before pregnancy:
• Associated Medical disorders: Anemia, control of diabetes or thyroid disorder.
• Surgical correction when indicated:
Commissurotomy or M valve replacement for MS, coronary bypass for ischemic heart or correction of cyanotic heart diseases.

Preconception Care
5-Prevention - postponing of pregnancy according to:
• The functional cardiac (Class III & IV NYHA)
• Risk of Maternal Mortality: moderate and severe (NYHA & ACOG \)
• Congenital heart disease risks in the fetus with affected family members

Risk of Maternal Mortality
Heart Diseases
Major Risk: Mortality 25-50%
• Pulmonary hypertension
• Aortic coarctation with valvular involvement
• Marfan syndrome with aortic involvement
Moderate Risk: Mortality 15-25%
Mitral stenosis, NYHA class III and IV
Aortic stenosis
Aortic coarctation without valvular involvement
Fallot tetralogy, uncorrected
Previous myocardial infarction
Marfan syndrome, normal aorta
NYHA = New York Heart Association & ACOG 1996

Risk of Maternal Mortality
Heart Diseases
Minimal Risk: Mortality 0-1%
• Atrial septal defect
• Ventricular septal defect
• Patent ductus arteriosus
• Pulmonary or tricuspid disease
• Fallot tetralogy, corrected
• Bioprosthetic valve
• Mitral stenosis, NYHA class I and II
NYHA = New York Heart Association&ACOG 1996

Management

Management According to the Functional Grade

Class I And II During Pregnancy
• Bed rest: At least 10 hours each night and half an hour after each meal.
• Light house work and walking is permitted but no heavy work.
• Avoid salt rich foods.
• Weight gain should not exceed 12 kg during pregnancy.
• Avoid contact with persons who have respiratory infections including common colds and flu condition.

Class I And II During Pregnancy
• Pneumococcal & influenza vaccines are recommended.
• Cigarette smoking is prohibited.
• Observe for signs and symptoms of deteriorating heart condition, if deterioration: Induce abortion< 13 weeks.
• Active treatment of anemia, hemoglobin should be kept at or above 12 g/dl throughout pregnancy.
• Tachycardia with MS can be treated with beta-blocker drug as propranolol (inderal) to have HR ± 80/m.
• Treatment of Paroxysmal Supra Ventricular (PSVT) & Atrial Fibrillation (AF).

Paroxysmal Supra Ventricular Tachycardia (PSVT)
• Symptoms include dizziness, syncope, chest discomfort, dyspnea and palpitations.
• Unilateral carotid sinus massage for 10 seconds. The patient should lie supine with IV fluids running and by ECG, if there is no response, try massaging the opposite side (not simultaneously).
• The patient may attempt a valsalva's maneuver during the carotid sinus massage. If this fails, the case is managed as with atrial fibrillation.

Atrial Fibrillation (AF)
Investigation for the precipitating causes:
• Myocardial ischemia
• Congestive heart failure
• Pulmonary embolism
• Fever
• Hypovolemia

Atrial Fibrillation (AF)
• Anxiety
• Hyper-thyroidism
• Symphathomimetic
• Aminophylline.
Any underlying causes or a medical disorder mandates treatment. Consultation with a cardiologist is of great value.

Atrial Fibrillation: Treatment
If the patient of AF is hemodynamically stable cardiac conversion is usually achieved by verapamil (e.g. Isopten) administered intravenously.
• If hemodynamically compromised or pharmacological conversion is not achieved direct current (DC) cardioconversion can be resorted to.
• Beta-blockers can be of help when the above measures are ineffective.
• Anticoagulation is necessary if history of TED.

Class I and II During Labor
• Semi-recumbent position with lateral tilt.
• Vital signs every 15 minutes and every 10 minutes in the second stage.
• Pulse rates > 100 and respiratory rates > 24 indicate impending heart failure.
• Oxygen by face mask + Pulseoximetry.
• Restriction of IV fluid to 75 ml per hour.
• Straining during the second stage of labor is avoided as far as possible.
• Outlet forceps or ventousse delivery can be used to shorten the second stage.

Class I and II During Labor
• No bolus oxytocin: sudden hypotension & no ergot (Methergin): sudden hypertension.
• Prophylaxis for the bacterial endocarditis -mainly streptococcus viridans - in parturient with RHD, valvular prosthesis, previous endocarditis, cardiac surgery and cyanotic heart D.
• Thromboprophylaxis: MS, HF, valvular prosthesis and other general risk factors
• Treatment of pulmonary edema (which is most likely developed immediately postpartum)

Antimicrobial Prophylaxis
• Either ampicillin, 2 g, or cefazolin or ceftriaxone, 1 g, is given intravenously. For penicillin-sensitive patients, one of the latter regimens is given, or if there is a history of anaphylaxis, then clindamycin, 600 mg is given intravenously.
• The recommended oral regimen is 2 g of amoxicillin. If enterococcus infection is of concern, vancomycin is also given.
American College of Obstetricians and Gynecologists (2008)

Treatment of Pulmonary Edema
Pulmonary edema is common with MS and most likely to develop immediately postpartum
• Propping up the patient to semi sitting position
• Oxygen by a face mask or nasal prong
• Furosemide (Lasix) IV 10 to 40 mg
• Morphine 5 mg IV slowly. If hypotension does not occur, 10 mg can be given 15 minutes later.

Class 1 and II During Labor
• Spontaneous vaginal delivery is associated with less morbidity in spite of the increased effort.
• Pain relief: Pethidine and gas & O2.
Epidural anesthesia (Fentanyl) is advised especially in primigravidas

Class I and II During Labor
Regional (spinal & Epidural) Vs General Anesth. Regional anesthesia is preferred to general anesthesia for CS. If hypotension occurs, IV infusion of 10 mg of metaraminol
(Aramine) in 250 ml of saline is the vasopressor of choice. It has a central inotropic action and is preferred to ephedrine as it does not cause tachycardia.
Regional anesthesia is not recommended in :
• A. stenosis: low or fixed cardiac output
• Tetralogy of Fallot
• Severe mitral stenosis: Pulmonary edema, as the resolution of the sympathectomy may coincide with the postpartum increase of venous return.

Class I & II During Puerperium
Continue close monitoring in the puerperium to avoid or to detect early the complications mainly:
• Infection
•Hemorrhage
• Thromboembolism
Breast feeding is allowed in the absence of heart failure.
Sterilization or other contraceptive options should be discussed.

Management of Class III & IV (Heart Failure)
• If seen early enough: termination is considered
• If the woman chooses to continue pregnancy, prolonged hospitalization or bed rest will often be necessary.
• Strict adherence to advice and treatment.
• Caesarean section is poorly tolerated.
• Treatment of heart failure.

Heart Failure
Heart failure is divided into 2 types:
• Left-sided heart failure is failure of the left ventricle. This may result in pulmonary edema. It manifests by dyspnea, orthopnea & paroxysmal nocturnal dyspnea
• Right-sided heart failure is failure of the right ventricle and may result in hepatomegaly, ankle edema, ascites and pleural effusion.

Treatment of Congestive Heart Failure (CHF)
Consultation with internist is usually necessary.
1. Recognition of the underlying cardiac disease.
2. Rapid correction of a precipitating cause like anemia, respiratory tract infection, administration of beta sympathomimetic drugs or tachyarrthmia.
3. Bed rest
4. Guard against thromboembolic complication. Exercises in bed and wearing compression stockings are of help, and heparin anticoagulation may be required.

Treatment of Congestive Heart Failure (CHF)
5. Diuretics: IV furosemide & Chlorothiazide (25 to 50 mg daily). The changes in the hematocrit and electrolytes should be monitored if use of diuretics is prolonged.
6. Digoxin is usually orally with a loading dose of 1.0 - 1.5 mg over 24 hours.
A maintenance dose of 0.125 to 0.375 mg daily
5. Vasodilators (↓ peripheral vascular resistance): sublingual nitroglycerine is the vasodilator of choice.

Surgical Management of RHD
This is better done before pregnancy.
The main indications in pregnancy are:
1) Pulmonary edema not responsive to medical management
2) A reliable history of previous pulmonary edema while under good medical management
3) profuse and uncontrollable hemoptysis
or
4) progressive pulmonary hypertension.

Surgical
• Management of RHD
When commissurotomy is the procedure of choice?
• Mitral valve is not calcified & no incompetence
• Symptomatic young women who are considering pregnancy.
Patients usually benefit for 5 to 20 years after commissurotomy.
If the symptoms recur later mitral valve replacement will be needed

Surgical Management of RHD
Mitral valve replacement
Operative mortality of 6%.
A porcine or human allograft (7-10 year duration) does not require chronic anticoagulant therapy and is recommended for women who desire to have other pregnancies.
A mechanical valve (life) is recommended for those not desiring children as it requires continuous anticoagulant therapy.

Rheumatic Heart Disease in Pregnancy

Rheumatic Fever
• The high prevalence rheumatic fever and RHDs in developing countries is due to the prevalence of streptococcal throat infection in children and crowdedness of the population.
• Rheumatic fever is an inflammatory autoimmune disease, as a delayed sequela of throat infection with group-A.

β hemolytic streptococci

.

Rheumatic Fever
• Rheumatic fever is manifested by damage to the collagen fibrils and the ground substance of connective tissue.
• The disease process is widespread and affects primarily the joints, the heart, CNS, skin, subcutaneous tissue and renal glomeruli.
• Active antibiotic treatment of throat infections, & tonsillitis will diminish the incidence of acute rheumatic fever.

Acute Rheumatic Fever (ARF)
ARF may be especially severe during pregnancy.
• Pregnancy tends to reactivate chorea (chorea gravidarum) and predisposes to its recurrence in subsequent pregnancy which can be fatal.
• The long-term prognosis is excellent if there is no acute carditis.
• Acute carditis: The sequela is Valvular infiltrations that is progressively replaced by fibrosis mainly MS & AR with liability of recurrences of ARF and additional cardiac damage.

Acute Rheumatic Fever (ARF)
• Treatment
• Bed rest
• Salicylates
• Glucocorticoids
• Penicillin therapy for any residual streptococcal infection.
• Prophlaxis: Deep IM injection of 1.2 million units of long acting (benzathine) penicillin / 4 weeks or erythromycin 250 mg orally twice daily.

Rheumatic Heart Disease in Pregnancy

Pathophysiology of Mitral Stenosis
• Rheumatic carditis cause progressive thickening, scarring, and calcification of the mitral leaflets and chordae.
• Fusion of the commissures and chordae decreases the size of the mitral opening.
• This obstruction results in the development of a pressure gradient across the valve in diastole and causes an elevation in left atrial and pulmonary venous pressures.

Pathophysiology of Mitral Stenosis
• Elevated left atrial pressures leads to left atrial enlargement, predisposing to AF & and arterial thromboembolism.
• Elevated pulmonary venous pressure results in pulmonary congestion and pulmonary edema.
• In advanced MS, patients develop pulmonary hypertension (due to P arteriolar vasoconstriction) and right-sided heart failure.

Symptoms & Signs of MS
• Dyspnea: Dyspnea is due to pulmonary congesion and reduced pulmonary compliance (capacity).
• Orthopnea & paroxysmal nocturnal dyspnea They occur as the disease progresses that result from augmentation of venous return from the dependent portions of the body upon recumbency.
• Coughing, hemoptysis (particularly at night) as a result of pulmonary congesion or pulmonary edema.

Symptoms & Signs of MS
Sequelae of left atrial enlargement :
• Premature atrial contractions
• Paroxysmal atrial tachycardia
• Atrial flutter, and atrial fibrillation (AF).
Sequelae of AF:
• Precipitate acute pulmonary edema or
• Predispose to intra-atrial thrombosis which can embolize to cerebral arteries
The jugular venous pressure ↑ when pulmonary hypertension develops
The radial pulse is typically ↓ in volume.

Symptoms & Signs of MS
Right ventricular hypertrophy
Shifting of the heart apex to the left in the 5 IS.
A diastolic thrill is usually felt over the apex.
Right ventricular failure (Late): Hepatomegaly, ankle edema, ascites and pleural effusion, particularly on the right side Auscultatation:
• Accentuated first heart sound
• An opening snap (OS) follows the 2nd
• Low pitched mid-diastolic murmur (D. Rumble), as a result of turbulent flow across the stenotic M valve

Chest X-ray: MS
•Enlargement of the left atrium
• Straightening of the left heart border.
•Prominent pulmonary trunk.
• Lung congestion or acute pulmonary edema (butterfly densities in the hilar region)
• In later stages right ventricular enlargement

Echocardiography of MS
The accurate diagnosis of stenosis: Mild MS< 2.5 cm2 Severe MS < 1cm² (Normal: 4.0 cm² )
It allows also assessment of:
• Pulmonary artery pressures
• Detection of other valve disease
• Visualization of left atrial thrombus

Mitral Regurgitation (MR)
• MR is leakage of blood from the left ventricle into the left atrium during systole
• It is frequently rheumatic and usually combined with MS (DM)
• May remain asymptomatic for years because the regurgitant load is well tolerated due to ventricular & atrial dilation.

• Mitral Regurgitation in Pregnancy
• Mitral regurgitation is well tolerated during pregnancy, probably due to decreased systemic vascular resistance
• Heart failure rarely occurs.

Mitral Valve Prolapse
• Occurring in 2.4%of the general population due to defect of collagen synthesis (genetic?)
• Systolic bulging of one or both mitral leaflets into the left atrium during systole caused by a defect in collagen synthesis
• Most patients with MVP are asymptomatic.
• Symptoms (rare): Atypical chest pain, dyspnea, palpitations and syncope
• A midsystolic click, with a late soft systolic murmur

Mitral Valve Prolapse in Pregnancy
• Rarely have complications.
• On the contrary, the pregnancy induced hypervolemia may improve the symptoms.
• Beta blockers may be given for palpitation.
• Mitral valve prolapse with significant mitral regurgitation: ↑ risk peripartum subacute bacterial endocarditis and should be given the prophylactic antibiotic regimen

Aortic Regurgitation (AR)
• It is the backward (diastolic) flow of blood from the aorta into the left ventricle.
• Causes: Rheumatic fever, connective tissue abnormalities, and congenital.
• Hemodynamic: Volume overload of the left ventricle and reduced diastolic perfusion of the coronary arteries.
• Symptoms: Mild cases of AR are

asymptomatic

Late or severe: sensation of carotid pulsation. If left ventricular failure develops: dyspnea, orthopnea and nocturnal dyspnea. Exertional chest pain (angina pectoris)

Aortic Regurgitation (AR)
• Physical signs
Water hammer" pulse (Corrigan pulse head bobbing, bounding carotid pulses, prominent capillary pulsation in the nail bed and pulsation of retinal arterioles. ↑ arterial pulse pressure . A diastolic thrill palpable along the left sternal border.
High-pitched diastolic murmur (left sternal )
• ECG : Tall R (L ventricular hypertrophy) and deep S waves over the right precordial leads.
• Echocardiograpby ( ± Transesophageal) Evidence of Left ventricular hypertrophy and dilation

AR in Pregnancy
Aortic regurgitation usually is tolerated quite well during pregnancy, and patients who are asymptomatic do not require any medical treatment.
If pulmonary congestion develops, restriction of activity is essential, and treatment with digoxin, diuretics, and vasodilators is indicated.

Aortic Stenosis (AS)
Stenosis reduces the normal 2 to 3 cm aortic orifice and creates resistance to ejection
• Causes: Rheumatic, Aging & congenital.
• Hemodynamic: Fixed low cardiac output in severe stenosis.
• Concentric left-ventricular hypertrophy.
• Symptoms: (Late) chest pain, syncope, heart failure, and sudden death from arrhythmias.
• Concentric left-ventricular hypertrophy

Aortic Stenosis in Pregnancy
• Clinically significant aortic stenosis is uncommonly encountered during pregnancy.
• Mild to moderate degrees of stenosis are well tolerated, but severe disease is life-threatening.
• Factors that aggravate the fixed cardiac output:
• Blood loss
• Regional analgesia
• Vena caval occlusion.
All of these aggravating factors decrease cardiac, cerebral, and uterine perfusion.

Valvular Heart Diseases
Mitral Stenosis: Presystolic murmur and opening snap.
• Mitral Regurgitation: Pansystolic murmur.
• Aortic Stenosis: Systolic ejection murmur
• Aortic Regurgitation: Diastolic murmur with wide pulse pressure
• Mitral Valve Prolapse: Systolic murmur with midsystolic click

Congenital Heart Diseases
The most common lesions seen in pregnancy are:
• Aortic Stenosis
• Atrial Septal Defect
• Ventricular Septal Defect

Congenital Aortic Stenosis
It is the most common congenital heart defect.
The manifestations of aortic stenosis due to bicuspid valve do not appear until relatively late in adult life
So, the condition is rare in reproductive age. The basic defect in aortic stenosis involves noncompliant valvular leaflets which place an increased blood volume load on the left ventricle during systole. The end result is ventricular hypertrophy and, later on failure.

Congenital Aortic Stenosis
• The syncope, angina pectoris and congestive heart failure
• A risk for sudden death that may be due to arrhythmias or sudden decrease in cardiac output due to the stenosis.
• Management includes bed rest, digitalis, diuretics and prophylactic heparinization for the duration of pregnancy
• Labor and delivery management: Hypotension and blood loss should be kept to the minimum. No conduction anasthesia

Atrial Septal Defect
• It is the second common congenital heart disease after congenital aortic valve stenosis. Many are asymptomatic until adult age.
• The defect results in slight to moderate left to right shunting of blood. The shunt is more marked if the septal defect is associated with mitral valve prolapse. Pulmonary hypertension is a rare sequela. A systolic murmur is heard on the left side of the sternum. It is tolerated during pregnancy. However, the defect is better being surgically corrected before pregnancy. Peripartum prophylaxis against SBE is required for all cases with atrial septal defect.

Ventricular Septal Defect
It results in left-to-right shunt. This can be significant if the defect is wider than the aortic orifice.
Arrhythmias are commonly associated.
A to-and-fro murmur is usually heard to the left of the sternum.
A marked shunt can result in pulmonary hypertension and reversal of the shunt, cyanosis or Eisenmenger syndrome.

VSD in Pregnancy
Pregnancy is well tolerated in uncomplicated cases.
Advanced cases should be strongly advised against pregnancy, and induction of abortion should be advised if recognized.
• The defect should be surgically corrected in children and if recognized before pregnancy, however antibiotic prophylaxis is recommended in the peripartum.

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Patent Ductus Arteriosus
• This is a common defect in adults, and the main effect is a left-to-right shunt. In the patient with an uncorrected patent ductus, pregnancy is usually well tolerated.
• However, with large shunt, pulmonary hypertension and reversal of the shunt occurs, which will markedly worsen the prognosis.

Tetralogy of Fallot
It is one of the most common forms of cyanotic heart disease in adults.
The primary cardiac defects are:
1- Intraventricular septal defect
2- Pulmonary valve or artery stenosis
3- Displaced aorta which overrides the ventricular defect
4- Right ventricular hypertrophy

Tetralogy of Fallot
• Pathophysiology: Right-to-left shunting secondary to the large ventricular septal defect and pulmonary artery stenosis, resulting in cyanosis.
• Uncorrected tetralogy of Fallot imposes a significant risk to both mother and fetus.
• Surgical correction improves maternal and fetal prognosis.
• Patient is susceptible to decreased venous return. Thus they are sensitive to:
• excessive blood loss
• regional anesthesia

Eisenmenger Syndrome
It consists of pulmonary hypertension and either right-to-left or bidirectional shunting through ASD, VSD or PDA.
• It is usually a long-term secondary sequela to a congenital cardiac defect that results in left-to-right shunt.
• The maternal and fetal prognoses are very bad.
• It cannot be corrected surgically.
• Termination of pregnancy at 1st trimester.
• Regional anesthesia should be avoided.

Coarctation of the Aorta
• It is uncommon in pregnancy
• There is a stenotic ring at the level of the left subclavian artery and is recognized by hypertension in the right arm only.
• The presenting symptoms may be chest pain or leg fatigue in severe cases.
• Collateral blood flow may be recognized by palpable pulsations or audible bruits over the ribs or notching of the inferior surface of the ribs on chest X-ray.

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Coarctation of the Aorta
• There is a risk of aortic dissection which may be caused by the hyperdynamic circulation of pregnancy, internal rupture or medial degeneration.
• They may develop rupture of a cerebral aneurysm.
• The coarctation is better surgically corrected before pregnancy.
• Management in pregnancy is mainly: Control of hypertension and Preventing bacterial endocarditis. Regional anesthesia is better.

Marfan Syndrome
• This autosomal dominant disease of connective tissue can result in significant complications including dissection of the aorta and aortic rupture.
• The defect is in fibrillin which is a constituent of elastin and is a general defect. However, aortic dilatation and dissecting aneurysm are the most serious complications and are more common during pregnancy.

Marfan Syndrome
• Women should be counseled that there is a 50% risk of their offspring inheriting this disorder. Those who choose to get pregnant should be carefully evaluated by echocardiography.
• Those with aortic diameters less than 40 mm have a small risk of aortic dissection.
• They should continuously receive beta-blockers (to ↓ pulse pressure) and almost complete bed rest and careful monitoring.

Marfan Syndrome
Those with aortic diameter > 40 mm are at high risk of aortic dissection and should be counseled to have first trimester termination or surgical repair of the aorta.

Peripartum cardiomyopathy (PPCM)
PPCM is a rare form of congestive heart failure occurring in the peripartum period. The classic criteria of PPCM are: 1-Cardiac failure in the last month of pregnancy or within 5 months of delivery.
2-Absence of a determinable etiology for cardiac failure.
3-Absence of demonstrable heart disease before the last month of pregnancy.
Persistence > 6 months postpartum carries a bad prognosis.

Peripartum Cardiomyopathy
Patient presents with symptoms and signs of congestive heart failure.
• Dyspnea is marked, other symptoms are orthopnea, precordial pain and cough.
• The hallmark finding is marked cardiomegaly
• ECG confirms increased end-diastolic dimensions.
• Therapy usually consists of digitalization, diuretic and low-dose heparin.

Ischemic Heart Disease
• Coronary artery disease which may lead to myocardial infarction is a rare in pregnancy.
• Patient may have the classical risk factors including familial history, smoking, obesity, familial dyslipidemia, hypertension, diabetes mellitus and thrombophilias (as antiphospholipid.)
• Diagnosis:↑ serum levels of the cardiac-specific contractible protein, treponin

Ischemic Heart Disease
• Pregnancy is usually inadvisable or should be delayed until coronary bypass.
• Treatment is as that of non-pregnancy
• Nitroglycerine and morphine are given
• Lidocaine: for malignant arrhythmia become indicated.

Ischemic Heart Disease
• Calcium channel blockers or beta-blockers are given if indicated.
• Tissue plasminogen activator can be given remote from delivery.
• If the infarct has healed sufficiently, vaginal delivery can be allowed.

Ischemic Heart Disease
• In some women interventional cardiac catheterization may become indicated. Coronary bypass surgery during pregnancy causes high risks.
• Echocardiography, radionuclide (thalium) studies and angiography. If there is no significant ventricular dysfunction, pregnancy will likely be tolerated.

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