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Philippine Heart Center Department of Nursing Education and Research

A Case Study on Ruptured Sinus of Valsalva, with Ventricular Septal Defect, with Severe Mitral and Tricuspid Regurgitation, Moderate Pulmonic Regurgitation and Moderate Pleural Effusion

Submitted in Partial Fulfillment of the Requirements For the 63rd Batch Post-Graduate Course in Critical Care Nursing Submitted to: Ma. Lilibeth Q. Icasiano, RN Course Coordinator

Submitted by: Carmelita O. Abulencia, RN Aprille Hershey R. Agustin, RN Amphi Joy A. Andaya, RN Paul Ryan I. Anido, RN Reigner Jireh D. Antiquera, RN Evan Harold Q. Antonio, RN Angela Socrates A. Baron, RN Arlene B. Basa, RN Charisse D. Belasa, RN Ju-en B.Blasurca, RN Ryan P. Bon, RN Rhonalyn S. Bravo, RN Elieza E. Bruzo, RN

May 28, 2010


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TABLE OF CONTENTS

I. II. III.

IV. V. VI.

Introduction Statement of Objectives Patients Profile a. Biographic Data b. History c. Prenatal and Post Natal History d. Gordons Health Pattern e. Physical Assesment Pathophysiology Course in the Ward Diagnostics and Laboratory Results

1 7 8 8 8 9 10 12 14 15

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Introduction Congenital Heart Defect (CHD) refers to a problem with the heart's structure and function due to abnormal heart development before birth. It is a broad term that can describe a number of different problems affecting the heart. The usual cause of a congenital heart disorders is failure of a heart structure to progress beyond an early stage of embryonic development. According to the Department of Health, approximately 8 out of 1,000 newborns are affected with Congenital Heart Defect. Congenital sinus of Valsalva aneurysm was first described 1839. This first published account describes rupture of a sinus of Valsalva, which is the most feared complication. Soon afterwards, clinicians described other cases of unruptured aneurysms and applied anatomic descriptions. The condition may have been clinically described for the first time in 1883. Aneurysm of a sinus of Valsalva is a rare congenital cardiac defect that can rupture, causing heart failure or other catastrophic cardiac events. If the aneurysm remains unruptured, it occasionally causes obstruction of cardiac flow resulting from compression of normal structures. Dissection of the aneurysm into the cardiac tissues may occur, causing obstruction or destruction of local structures. Sinus of Valsalva aneurysms are uncommon clinical entities but are known to be associated with up to 0.5-1.5% of patients. They develop as a result of a separation of the aortic media of the sinus from the ventricular fibrous structures leading to a progressive protrusion into the low-pressure chamber due to the pressure differential. Most often the aneurysm begins in the right coronary sinus (80%) and terminates in the right ventricle (72%) but up to 15% of cases originate in the non-coronary sinus, which usually leads to a termination in the Right Atrium. Sinus of Valsalva aneurysms can be congenital or acquired as a result of injury, infection, or be related to Marfan syndrome or Behcet disease. Up to 47% of patients with sinus of Valsalva aneurysms are found to have an associated congenital defect. In one series an associated ventricular septal defect was most common (49.3%), followed by aortic insufficiency (23.4%), tricuspid regurgitation, infundibular pulmonary stenosis, patent ductus arteriosus and patent foramen ovale (each 1.9%). An association with bicuspid aortic valves and aortic coarctation has also been described. A ventricular septal defect (VSD) is a defect in the septum between the right and left ventricle. The septum is a wall that separates the hearts left and right sides. Septal defects are sometimes called a hole in the heart. Its the most common congenital heart defect in the newborn; its less common in older children and adults because some VSDs close on their own. Tricuspid regurgitation is leakage of blood backward through the tricuspid valve each time the right ventricle contracts. Tricuspid regurgitation usually results when the right ventricle enlarges and resistance to blood flow from the right ventricle to the lungs is increased. Resistance may be increased by a severe, long-standing lung disorder, such as
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emphysema or pulmonary hypertension, by disorders involving the left side of the heart, or rarely by narrowing of the pulmonary valve (pulmonic stenosis). To compensate, the right ventricle enlarges, stretching the tricuspid valve and causing regurgitation. Tricuspid regurgitation affects about 4 out of 100,000 people. It may be found in those with a type of congenital heart disease called Ebstein's anomaly. Mitral regurgitation is a disorder of the heart in which the mitral valve does not close properly when the heart pumps out blood. It is the abnormal leaking of blood from the left ventricle, through the mitral valve, and into the left atrium, when the left ventricle contracts, i.e. there is regurgitation of blood back into the left atrium. The mitral valve is composed of two valve leaflets, the mitral valve annulus (which forms a ring around the valve leaflets), the papillary muscles (which tether the valve leaflets to the left ventricle, preventing them from prolapsing into the left atrium), and the chordae tendineae (which connect the valve leaflets to the papillary muscles). A dysfunction of any of these portions of the mitral valve apparatus can cause mitral regurgitation.The most common cause of mitral regurgitation is mitral valve prolapse (MVP), which in turn is caused by myxomatous degeneration. The most common cause of primary mitral regurgitation in the United States (causing about 50% of primary mitral regurgitation) is myxomatous degeneration of the valve. Myxomatous degeneration of the mitral valve is more common in females, and is more common in advancing age. This causes a stretching out of the leaflets of the valve and the chordae tendineae. The elongation of the valve leaflets and the chordae tendineae prevent the valve leaflets from fully coapting when the valve is closed, causing the valve leaflets to prolapse into the left atrium, thereby causing mitral regurgitation. Pulmonic regurgitation, also known as pulmonary regurgitation, is the backward flow of blood from the pulmonary artery, through the pulmonary valve, and into the right ventricle of the heart during diastole. While a small amount of pulmonic regurgitation may occur in healthy individuals, it is usually detectable only by an echocardiogram and is harmless. More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants further investigation. Pleural effusion is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. Excessive amounts of such fluid can impair breathing by limiting the expansion of the lungs during inhalation. Pulmonic regurgitation, also known as pulmonary regurgitation, is the backward flow of blood from the pulmonary artery, through the pulmonary valve, and into the right ventricle of the heart during diastole. While a small amount of pulmonic regurgitation may occur in healthy individuals, it is usually detectable only by an echocardiogram and is harmless. More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants further investigation.

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Figure 1: Illustration of a Ruptured Sinus of Aneurysm

Figure 2: Illustration of a Ruptured Sinus of Aneurysm Figures show the TEE and Doppler examination. The 4 chamber view at 0 degree (Panel A) shows an aneurysmal sac protruding into the right atrium from the aortic root (open white arrows). Green arrows indicate tricuspid valve. Color Doppler shows turbulent flow (solid white arrows) from the aorta through the pouch into the right atrium (Panel B). The same saccular-like structure in the RA is seen in short axis view at 59 degrees with turbulent flow by color Doppler (Panels C & D). Interrogation by CW Doppler revealed continuous turbulent flow in systole and diastole (Panel E).

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Figure 3: Ventricular Septal Defect

Figure 4: Mitral Regurgitation

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Figure 5: Tricuspid Regurgitation

Figure 6: Pulmonary Regurgitation

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Figure 7: Pleural Effusion

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Statement of Objectives Upon accomplishment of this case study, the participant should be able to: 1. Recognize and understand the disease condition; 2. Identify predisposing and precipitating factors that could possibly contribute to the occurrence of the disease; 3. Understand the normal anatomy and physiology of the affected organs that are affected by the underlying disease condition; 4. Identify specific theoretical causes and clinical manifestations, and trace the pathophysiology of the involved disease entity; 5. Identify nursing problems and construct nursing care plans specifically; 6. Identify ways of preventing the occurrence of the disease or problem by providing health teachings upon the recommendation of the study.

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Patients Profile

I.

Biographic Data Name: Dora Age: 12 years old Date of Birth: 10/13/1997 Sex: Female Address: Alaminos, Laguna Religion: Roman Catholic Marital Status: Single Occupation: Student Chief Complaint: Nahihirapan po ako huminga at masakit po dibdib ko Diagnosis: Ruptured Sinus of Valsalva and Ventricular Septal Defect with Severe Tricuspid and Mitral Valve Regurgitation, and Moderate Pleural Effusion.

II.

History A. Past Health History An interview with the mother was done to obtain the patients past health history. Dora is the third child among the 6 siblings, who was born, term via normal spontaneous vaginal delivery at home by the local hilot or midwife. The patient was noted to be small for gestational age. Obstetrics history as per the mothers information shows no consistent prenatal checkup for all six offspring. The mother states that for the patient specifically, she only went on her third trimester to have a checkup. The mother also added that she usually use birth control pills but would often miss a dose. During infancy, the patient was noted with diaphoresis when feeding, but no other symptoms were noted. After the third month of life, the patient was left under the care of her paternal grandparents. As claimed, there was no history of recurrent respiratory tract infections. The mother however noted that her daughter had poor weight gain around that time. She also stated that she cannot remember if her daughter had any vaccinations, except for polio given at the local community center. The patient stayed with her grandparents until the age of 9, and moved back to her parents when the former passed away.

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B. History of Present Illness A month and a half prior to her admission, Dora experienced fever and cough. By the end of March, the mother reported that her daughter had episodes of dyspnea, easy fatigability, on and off fever, and progressive two pillow orthopnea. She also had episodes of bipedal edema, which on some days are present and will disappear in a matter of weeks. This prompted the mother to bring her to the local physician, who suggested to have a 2D Echo done. The result of the test was: Ruptured Sinus of Valsalva, Severe Tricuspid Regurgitation, Severe Mitral Regurgitation, Mild Pleural Regurgitation, Moderate Pleural Effusion and Mild Pulmonary Hypertension. They were advised to seek consult at Philippine Heart Center but were not able to comply due to financial constraints. No other medications were taken and no other consults were done. Persistent episodes of dypsnea, and chest plain prompted consult at Philippine Heart Center. C. Prenatal History The mother is currently 43 years old with an obstetric scoring of Gravida 6, Para 6 with three Term, and three Preterm. She was 31 years old when she gave birth to the patient. All were delivered via normal spontaneous vaginal delivery at home with the local hilot practitioners. She decided to practice artificial family planning on her second child using birth control pills being given at the local community health center, but was not able to follow her schedule properly. The mother admits that her third child up to the sixth were results of missed doses. She does not remember any specific vaccinations given to her during her childhood. The mother also states that she does not attend any prenatal checkup and would usually rely on local practices and procedures. Post Natal History The patient was born term, with no noted abnormalities. The patient has not undergone any newborn screening. The mother recalled she had breastfed the patient for a month before she was transferred to her grandparents. Foods were introduced beginning on the sixth month. The mother stated that the patient seems too small on her sixth month. Regular vaccinations were not followed. At 10 years old, the patient began manifesting size of easy fatigability, dyspnea and generalized weakness. Episodes of dypsnea and fatigability would usually manifest when she engages herself in high levels of activity. When symptoms became present, they are usually addressed by rest, and sleep in a side-lying position. Two months prior
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to admission, symptoms were becoming more frequent, thus prompting the parents to seek consultation for their daughter D. Familial History The patient states that both her parents were diabetic and hypertensive and the same applies for her husband. Coronary Artery Diseases were also present at the patients father side. The mother also noted that there is also a presence of a congenital heart defect on the patients father side. However, the mother is not aware of the specific defect. No other siblings have manifested any symptoms indicating congenital heart problem, aside from the patient being presented. E. Gordons Health Pattern A. Health Perception and Health Management Pattern The patient understands that she is not well, and she is willing to cooperate so that she can get better. The mother of the patient also understands her daughters condition and would like to be a part of her health management in any way possible. B. Nutritional and Metabolic Pattern The patient states that her usual diet contains rice and vegetables, specifically kangkong or water spinach. On some occasions, she is able to eat chicken and pork. She does not take any supplemental vitamins. The patient is at present, 21 kilos. C. Elimination Pattern The mother recalls that prior her daughters admission, her daughter is urinating and voiding well, usually 2 to 3 times a day. The patient did not notice anything significant in her elimination as well. D. Activity-Exercise Pattern The patient states that her usual activity months prior to her admission involves doing household chores, going to school, and helping out her parents sell figurines. She used to play together with kids of her own age, not until a month prior to her admission when it became difficult to breathe and she gets tired easily. E. Sleep-Rest Pattern The patient stated that she gets around 6 to 7 hours of sleep. A month prior to her admission, she stated it became difficult to sleep due to shortness of
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breath, which is usually resolved by lying on her left side and by using pillows. Two days prior to her admission, she hardly gets any sleep at all due to episodes of chest pain. F. Cognitive- Perceptual Pattern The patient has a good sense of vision, hearing, taste, touch and smell. She has no difficulty talking or remembering things. She is also oriented to time and place. G. Self-Perception and Self-Concept Pattern The patient is concern about her current hospitalization and states that she is afraid of the outcome of her present hospitalization and the implications of any treatment in her future activities. During the interview, the patient cannot maintain good eye contact and would often hesitate expressing herself verbally. H. Role-Relationship Pattern Dora is the third child among the six siblings. Her mother and father are both Highschool graduates, and earn their daily living by selling figurines at a public market nearby. The mother stated that there are no issues regarding her family, and they are satisfied with what they currently have. She admits that on some occasions, conflict would arise due to financial reasons but gets easily resolved as well. I. Sexuality-Reproductive Pattern The patient has not experienced her menarche. J. Coping-Stress Tolerance Pattern The patient stated that by watching television and doing some activities such as coloring books, playing jack stone would usually lessen the pain. Her mother is supportive as well, and is always present whenever she has shortness of breath or an episode of pain begins. K. Value-Belief Pattern The patient and her family are all Roman Catholic who occasionally attends Sunday services. The mother stated that their current socio-economic condition has made them closer to their faith.

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Physical Assessment (Done on May 17, 2010 5th day in the Pediatric Intensive Care Unit) A. Vital Signs Heart Rate: 126 beats per minute Respiratory Rate: 32 breaths per minute Temperature: 37.8 C Blood Pressure: 120/30(Arterial Line) 110/0 BP cuff Weight: 21 kg Height: 150 cm Allergies: No known allergies B. Central Nervous System Dora is awake, ambulatory and coherent with a Glasgow Coma Scale of 15 (Eye Response: 4, Verbal Response: 5, Movement: 6). No alteration in sensory status. C. Cardiovascular System The patient has an abnormal heart sound (thrill) heard at the 6th Intercostal Space Left anterior axillary line, with regular heart rhythm. The patient still presents Sinus Tachycardia. She also presents with dynamic precordium and suprasternal pulsation. The upper and lower peripheral pulses are also present. The sclerae are white, and she also has a pink palpebral conjunctivae. Capillary refill is 2 3 seconds. D. Respiratory System The patient has an irregular breathing pattern with subcostal retractions and dyspnea. There is a decreased breath sound in the left lower lung, as well as the presence of crackles in the bibasal lung fields. The patient also prefers to sit up as this helps her breathe easier. Uses of accessory muscles were also noted. E. Gastro-Intestinal System The patient has a flabby abdomen, with normoactive bowel sounds ranging from 8 to 10 sounds in each quadrant. She also has a regular bowel pattern, with one bowel movement in the morning and another in the evening. F. Urinary System The patient has a normal urine pattern with no bladder distensions. The 7 am to 3 pm Intake and output shows an output of 800 cc, whereas the input on the same timeline was noted at 400 cc. The color of the urine is yellow; however, lab results revealed

BMI: 9.33 (Underweight)

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that there are traces of blood in the urine graded as +2, indicating hematuria and traces of protein, graded as +2, indicating proteinuria. G. Musculoskeletal System The patient presents generalized body weakness and looks cachexic. She also presents grossly normal extremities with no deformities. The patient also scores a 3 out 5 in muscle strength in all extremities, with good coordination. The patient needs some assistance in performing her activities in the hospital. H. Integumentary System The patient presents poor skin turgor and dry lips. No other deviations were observed. I. Psychosocial and Cognitive Development Under Ericsons Psychosocial Development, Dora is under the Identity vs. Role Confusion and under Piagets Cognitive Development, she is under the Formal Operational Stage. Under the former, she has good interpersonal relationship with her parents as well as her siblings and friends. She misses her friends as well her siblings. Even though there are some restrictions due to her health condition, she still finds time to do her household chores as well as her studies. Under the latter, she enjoys doing different tasks such as coloring, reading, as well as answering mathematical equations.

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Pathophysiology
Non-Modifiable Factors: Genetics: As stated by the mother, there is history of Congenital Heart of the patients father side. Race: Asians (Filipino) Shows increased to acquire Aneurysm of the Sinus of Valsalva. Age: 11 67 years old (Patient is 12 years old). Aneurysm and Rupture increases within this age group. Modifiable Factors: Exposure to Teratogens: Father is an active smoker (3 packs per day) and exposure to passive smoking during pregnancy can increase the risk. Alcohol drinking of the mother may also contribute to the development of birth defects. Lifestyle: Attitude towards prenatal and newborn checkups was not prioritized. Health-seeking behavior such as knowledge about vaccinations and family planning may also contribute as well.

Organogenesis Development of the heart septum and valves occurs on the 6th and 7th week of pregnancy.
Endocarditis Neutrophil: 42% and high lymphocytes: 48% (due to acitive infectious process: endocarditis), low Eosinophils: 1% ESR 40 mm/hr

Formation of weak aortic structures

Ventricular septal defect

Highly turbulent blood flow causes endothelial damage which predisposes to the creation of lesions

Increased susceptibility of infection and migration of bacteria to the heart, which causes inflammation.

Strain of aortic pressure. Aortic medial is defective and fragile. The sinus gradually weakens.

Aortic Insufficiency (widened pulse pressure)

Separation of the media from the attachment of the leaflets, results into aortic media retraction.

Allows progressive dilatation of the sinus eventually leads to Aneurysm of the Right Sinus .Continues Filling and stagnation of blood. Aneurysm protrudes to the right atrium and right ventricle.

This causes obstruction on the ventricular outflow tract. Instability and progressive distortion of the right sinus leads to the compression of the chambers of the heart. Alteration in electrical activity of the heart (SVT (on the ECG reading on the 13th of May, 2010 ,and Sinus Tachycardia with episodes of PVCs soon afterwards) The progressive increase pressure on the right side of the heart, eventually to the rupture of the sinus.

Sudden gush of blood will affect all chambers of the heart

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Effects on the Right side of the heart: Tricuspid valve regurgitation and Pulmonic Regurgitation Signs and Symptoms: Jugular vein distention, Bipedal edema, increase blood pressure, increase pressure on the veins of the nasal area that leads to epistaxis (during hospitalization)

Effects on the Left side of the heart: Mitral Regurgitation. Increase pressure and volume will lead to backflow from Left atrium to Pulmonary veins to the lungs, leading to pleural effusion. Signs and Symptoms: Crackles and Difficulty of Breathing (upon admission)

Progressive congestion leads to increased cardiac workload, thus leading to ineffective contractility of the heart.

Multi chamber hypertrophy Decreased Cardiac Output

Decreased Circulation to the Brain: Signs and Symptoms: Occasional headache, Restless, syncope

Decreased Circulation to the Kidney: Signs and Symptoms: Altered function of the kidney proteinuria and hematuria

Altered nutrition and decrease in appetite increases the risk and susceptibility to infection. Easy fatigability due to decrease energy source. Signs and Symptoms: BMI: 15.3

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Narrative Pathophysiology

The Ruptured Sinus of Valsalva is caused by modifiable factor such as exposure to teratogens (father is an active smoker consuming 3 packs per day thus exposed to passive smoking during pregnancy and non-modifiable factors namely; genetics (as stated by the mother, there was a history of Congenital Heart Defect paternal side), race (asians shows increased to acquire Aneurysm of the Sinus of Valsalva), Age aneurysm and Rupture increases within 11-67 age group). These factors contribute to the malformation of heart structure during the organogenesis period specifically on the 6th to 7th week of pregnancy .Generally, the defect is focused at the aortic structure specifically the sinus which is one of the anatomic dilations of the ascending aorta, which occurs just above the aortic valve and is associated with ventricular septal defect. Aneurysms typically develop as a discrete flaw in the aortic media within one of the sinuses of Valsalva. Under the strain of aortic pressure, the sinus gradually weakens and dilates, causing the formation of an aneurysm. Lack of supporting tissue (eg, ventricular septal defect) may contribute to instability and progressive distortion of the aortic sinus, often with associated aortic insufficiency. Deficiency of the aortic media where it attaches to the aortic annulus produces dilation of the aortic sinuses over many years. Distortion and prolapse of the sinus and aortic valve tissue leads to progressive aortic valve insufficiency as manifested by widened pulse pressure. Continues filling and stagnation of blood predisposes the aneurysm to shift to the right ventricle then the right atrium. This will lead to obstruction of the right ventricular outflow and causes further compression. It will lead to altered electrical activity of the heart as manifested by sinus tachycardia, Supraventricular tachycardia, Atrial Fibrillation, Premature Ventricular Contractions and Ventricular Tachycardia on clients ECG strip. Furthermore, it increases pressure on the Right and Left side of the heart. With this case, the right portion would have the initial affectation. First, the force exerted results to the rupture of the sinus. There would be a sudden gush of blood that causes the pulmonary and tricuspid valve to regurgitate. On the other hand, the left side wound manifest mitral valve regurgitation and further destruction of the aortic valves happens. The pressure also causes the backflow of blood to the left atrium to the pulmonary system thus formation of crackles and fluid shift to the pleural space occur. As the manifestations progress, cardiac workload also increases, ineffective contractility takes place and multi-chamber hypertrophy happens thereby leading to cardiomegaly. This causes compression of the diaphragm and becomes a contributory factor to the development of dyspnea, Further to this, decreased cardiac output in systemic circulation occurs. In order for the body to compensate tachypnea and tachycardia are manifested. Since oxygenation is compromised, two main body structures namely the brain (headache and syncope) and kidneys (proteinuria and hematuria) are affected. On the other hand, the highly turbulent blood flow in the ventricular septal defect causes endothelial damage which predisposes to the creation of lesion that increases
63rd Critical Care Course A Case of Ruptured Sinus of Valsalva

susceptibility of infection and migration of bacteria to the heart. Inflammation on the endometrium (endocarditis) causes fever and increased WBC. Moreover, the hypermetabolic state occurs as well as insensible fluid loss causes increase in energy consumption. This results to easy fatigability, powerlessness, and activity intolerance. Appetite would also be decreased with the additional depression of the satiety center as evidenced by weight loss, low body mass index and cachexia. To further confirm the disease process, different lab examinations were conducted which revealed low HgB: 113g/L, HcT: 0.36, MCH: 25.5 pg, and MCHC: 318 g/L (related to decreased blood supply, active blood loss due to epistaxis, hematuria and nutritional imbalance). Low Neutrophil: 42% and Eosinophils: 1%,high lymphocytes: 48% , platelet count: 520 monocyte 8%, ESR: 40mm/hr and CRP: 51.4 mg/ml are due to the inflammatory and infectious process in the endometrium. Lastly, because of the congestion and the rupture, chest x-ray and 2DECHO revealed valvular and heart structure problems.

63rd Critical Care Course A Case of Ruptured Sinus of Valsalva