Origin of the basal systolic murmurs in mitral stenosis. A study with intracardiac phonocardiography

In order to study the origin of the basal systolic murmurs in mitral stenosis, left and right heart catheterization was performed in 18 patients with mitral stenosis using intracardiac phonocardiography. Our data revealed that the basal systolic murmurs originated in the aorta, the pulmonary artery, and the outflow tract of the right ventricle.In 14 cases, we noted the maximal ejection systolic murmur in the aorta near the aortic valve. However, in two cases, there was a loud systolic murmur in the pulmonary artery. These murmurs occurred in early to mid-systole and were crescendo-decrescendo in configuration. The pitch of the murmur ranged from low to medium frequency in the majority of cases. They are produced by the turbulence of blood flow in the aorta and the pulmonary artery.A late systolic murmur was also recorded in the outflow tract of the right ventricle in two patients. This is thought to occur due to functional or relative infundibular stenosis of the right ventricle. It differs in location and timing from those in the aorta and the pulmonary artery. The outflow tract of the right ventricle is regarded as the third origin of the basal systolic murmur in mitral stenosis.     

Traumatic right coronary arterial-right atrial fistula

A healthy 25 year old man presented with a machinery murmur and a history of a penetrating thoracic injury. Hemodynamic and angiographie evaluation demonstrated a fistula from the right coronary artery to the right atrium that was later successfully repaired. This case and similar reported cases indicate that fistula formation is a late complication of penetrating thoracic injuries. The onset of the murmur occurs with enlargement of the fistula and typically is delayed for several weeks to months after the initial injury.     

Right coronary arteriocameral fistula into the right ventricle. An inspiratory accentuation of continuous murmur: a case report.

A case of a right coronary artery fistula into the right ventricle is presented. The clinical diagnosis was based on the presence of a continuous murmur best heard in the epigastrium, which increased in intensity and duration on inspiration . This report stresses the importance of this sign in the localization of the arterial fistula to the right ventricle. To the best of our knowledge, there are no previous reports of this sign.     

Non-invasive diagnosis of a right coronary artery-right ventricle fistula by Doppler echocardiography]

A six-year-old girl with an atypical continuous precordial murmur, was suspected of having a coronary arteriovenous fistula. A markedly dilated right coronary artery was revealed by two-dimensional echocardiography. The pulsed Doppler examination showed a diastolic retrograde flow in the ascending aorta, with a normal flow in the left ventricular outflow tract. With the sample volume in the right ventricle a continuous turbulent flow was observed. Color flow mapping showed a turbulent, systolic-diastolic flow in the right ventricle. The fistula was confirmed by cardiac catheterization.     

Ruptured aneurysm of the sinus of valsalva coexisting with a ventricular septal defect and single coronary artery.

A 26-year-old man had been diagnosed with a cardiac murmur from birth. In 1998, he was admitted to hospital because of slight fatigue. A grade 5/6 continuous murmur was audible near the right sternal border at the second intercostal space. Doppler echocardiography detected an abnormal flow that suggested that an aneurysm of the right coronary sinus of Valsalva had ruptured into the right ventricular inflow tract. Blood tests showed a 19% step-up in oxygen saturation value between the right atrium and right ventricle, indicating a ventricular septal defect with left to right shunt. Coronary angiography revealed a single coronary artery. Surgical repair was carried out and the patient made an uneventful recovery. This rare combination of a ruptured aneurysm of the sinus of Valsalva coexisting with a ventricular septal defect and a single coronary artery has not been reported previously.     

Fistula of the right coronary artery near the right ventricle. An echocardiographic study

A case of a 42-year-old woman with a right coronary artery fistula into the right ventricle is reported; she was asymptomatic and a continuous precordial murmur was found on physical examination. We discuss the usefulness and limitation of the Echo 2-D/Doppler findings, concerning this diagnosis, which was only definitively established by angiocardiography.     

Ruptured aneurysm of the sinus of Valsalva into the right ventricle--apropos of a case

A 37 year old man was referred to our institution because of a cardiac murmur, exertional dyspnea and fatigue, symptoms that began since 18 years of age. He reported a cardiac murmur since childhood, with no past history of rheumatic fever or infectious endocarditis. On clinic examination there was a systolic-diastolic murmur louder in the third and fourth left intercostal space, just at the sternal left border. The 2 D-echo revealed a small disruption in the aorto-septal continuity. Right heart catheterisation was performed, showing an increased pressure in the pulmonary artery and right ventricle; an increase in the oxygen saturation on the right heart chambers, suggested the presence of a left-to-right shunt, nevertheless the exact location of the defect was not possible to recognise. The study was complemented with Doppler color flow imaging that revealed a turbulent flow through the defect, with blood flowing from the aortic root into the right ventricular outflow tract. The diagnosis of ruptured aneurysm of sinus of Valsalva was made, being confirmed later by aortic angiography. A rare case is reported in which an aneurysm of the right coronary sinus ruptured into the right ventricle; we emphasize the important contribution of the Doppler color flow imaging to the correct diagnosis, technique rarely described in this type of complication.     

Acquired systemo-pulmonary fistula after pleurectomy responsible for a continuous thoracic murmur

Systemo-pulmonary fistulae are rare. The case of a 27 year-old man, hospitalized for exploration of a continuous thoracic murmur, is reported. A right pleurectomy had been performed 2 years previously because of a recurrent spontaneous pneumothorax, and no murmur was present at that time. Angiography showed a systemo-pulmonary fistula with the right internal mammary artery and branches of the right axillary artery as afferent vessels, and the right pulmonary arteries and veins as efferent vessels. Blood gases measurements demonstrated a left-right shunt. The acquired nature of the fistula was suspected because of the history of right pleurectomy and the acquired nature of the murmur. There was no indication for surgery because of the complexity of the fistula and the absence of symptoms.     

Primary coronary fistulas. Angiographic study of 21 cases

A retrospective study of 21 cases of congenital primary coronary fistulae and a review of the literature underline the exceptional features of 2 of our cases and recall the main features of coronary fistulae. Coronary angiography (18 cases) or aortography (3 cases) confirmed the diagnosis in our 21 cases. Six of them involved the right coronary artery, 12 the left one, and 3 the two coronary arteries. Seven of them involved the right heart (2 right atrium, 5 right ventricle), 11 the main pulmonary artery, 2 the left heart (the left ventricle in both cases), and the last case was a coronaro-bronchial fistula. Our series comprised 11 men and 10 women; the mean age was 33 (range: 4 days to 67 years of age). The main presenting features were: murmur (7 cases), angina (7 cases), angina (7 cases), Stage II or III dyspnoea (5 cases). Clinical examination showed a systolic-diastolic murmur in 12 cases and a systolic murmur in one case. The chest X ray showed signs of left to-right shunt in 6 cases. The right heart catheterisation of 16 of the 19 left-to-right shunts confirmed the shunt in 9 cases. The 7 cases of coronaro-right cardiac fistulae drained directly (4 cases), by aneurysmal dilatation with diaphragm (3 cases); the 2 cases of coronaro-left-ventricular fistulae drained via microfistulae; the 11 cases of proximal coronaro-pulmonary fistulae drained mostly by an angiomatous plexus (9 cases).(ABSTRACT TRUNCATED AT 250 WORDS)     

Penetrating injury of the chest and coronary arteriovenous fistula.

An unusual case of coronary artery fistula, due to a stab wound in the chest in a 17-year-old male patient, with a resultant acute inferior myocardial infarction is presented. At surgery, complete transection of the right coronary artery was found, which formed a fistulous communication with both the right atrium and the right ventricle. The development of a continuous murmur in the period immediately after the injury, the absence of hemopericardium, and multiple cardiac-chamber involvements were unique features of the case. The inferior myocardial wall was akinetic due to infarction and did not improve following revascularization surgery. A brief review of the literature is also presented.     

An elephant trunk appearance—what is the diagnosis?

Coronary cameral fistula is a rare congenital cardiac abnormality. An 18‐year‐old boy presented with features of right heart volume overload. Clinical examination was suggestive of hyperdynamic circulation with continuous murmur in precordium. 2D echocardiography showed dilated right coronary artery, and 3D echocardiography added information in tracing the track of the fistula which was consistent with the diagnosis of right coronary cameral fistula draining into the right ventricle. Coronary angiograms revealed an unusually dilated right coronary artery giving the appearance of an “elephant trunk” and with a fistulous tract into the right ventricle. Considering the higher risks of surgery in such difficult cases, we performed a successful transcatheter closure of the fistula using an Amplatzer vascular plug.     

Abnormal implantation of a coronary artery in a 53-year-old patient. Surgical correction

In a 53-years old patient complaining of dyspnoea and angina of effort, the coronary arteriography demonstrated ab abnormal implantation of the pulmonary artery into the left coronary-artery. Correlated with the 39 cases of the adolescent and the adult published in the literature, the case reported has some peculiarities: poorness of the auscultatory signs consisting in a mild apical systolic murmur; electrocardiographic pattern of left complete bundle-branch block; presence of massive calcifications visible by X-ray into the lateral, wall of the left ventricle. Coronary arteriography and catherization made it possible to demonstrate a left-to-right shunt by backward-flow revascularization of the left coronary artery starting from the right coronary artery. A simple suture of the implantation foramen of the abnomal coronary artery resulted in increased pressure into this artery and was followed by disappearance of angina during an observation period of 5 months.     

Spontaneous occlusion of a congenital coronary artery fistula

Cardiac catheterisation of a 6-month-old asymptomatic infant with a continuous precordial murmur revealed a congenital coronary artery fistula draining into the right ventricle. Following a period with no complications and the disappearance of the cardiac murmur, recatheterisation at the age of 4 years showed spontaneous complete closure of the fistula. This case in connection with 3 similar cases from the literature suggests that in asymptomatic patients with a congenital coronary artery fistula surgery might be delayed until school age in order to give spontaneous closure a chance.     

Fibroplastic parietal endocarditis with eosinophilia.

A 44-year-old woman with marked eosinophilia, leukocytosis, congestive heart failure, and the murmur of mitral stenosis had a restrictive type of pulse contour at cardiac catheterization. A right atrial angiogram revealed a huge right atrium, a small right ventricle, and a dilated contractile outflow tract consistent with the diagnosis of Loeffler's endocarditis. A marked conduction delay at the atrial level was demonstrated by His bundle electrogram studies.     

A case of adult pulmonary supravalvular membranous stenosis which was successfully treated by percutaneous transluminal balloon dilatation]

We report a case of adult pulmonary supravalvular stenosis which was successfully treated with percutaneous transluminal balloon dilatation (PTBD). A 42-year-old man was admitted for a heart murmur and abnormal findings in the screening chest roentgenogram. Having no symptoms on admission, his physical activity was evaluated as Class I according to the classification of the New York Heart Association. A systolic murmur (Levine III/VI) with split second sound was audible at the left sternal border in the 3rd intercostal space. Chest roentgenogram revealed 57% of the cardio-thoracic ratio and no signs of increased pulmonary vascular markings. ECG showed incomplete right bundle branch block. Echocardiography and right ventriculography visualized the supravalvular membranous structured stenosed pulmonary artery 1cm above the pulmonic valve. Systolic pressure gradient between the right ventricle and the distal main pulmonary trunk was 54mmHg. The patient was diagnosed as having pulmonary supravalvular membranous stenosis. PTBD was applied using Inoue balloon catheter, where inflation was initiated from approximately 120% of pulmonary arterial diameter and terminated at 150%. This procedure decreased systolic pressure gradient from 54mmHg to 36mmHg without complication. After PTBD right ventriculography demonstrated that a part of the membranous structure had become floppy and movable. Pulmonary arterial diameter was unchanged. We considered that PTBD tore the membranous structure, which consequently resulted in the decrease of systolic pressure gradient. Systolic murmur diminished to Levine II/VI. Six months later, cardiac catheterization showed no change in systolic pressure gradient compared with that immediately after PTBD. This is the first report on pulmonary supravalvular membranous stenosis successfully treated with PTBD.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ruptured aneurysm of the sinus of Valsalva with Wildervanck syndrome (cervico-oculo-acoustic syndrome), blepharoptosis and short stature: case report.

A 62-year-old woman was admitted to hospital because of nausea. A grade 5/6 continuous murmur was audible near the left sternal border at the second intercostal space. Chest X-ray showed cardiomegaly and bilateral pleural effusion. She was diagnosed as heart failure and a diuretic was prescribed. After remission of the heart failure, echocardiography showed shunt flow from the right coronary cusp to the right ventricle. Aortography revealed that an aneurysm of the right coronary sinus of Valsalva had ruptured into the right ventricle. Coronary angiography revealed a single coronary artery. Chest computed tomography revealed persistent left superior vena cava. Surgical repair was carried out and the patient made an uneventful recovery. In addition to these cardiovascular abnormalities, she had Wildervanck syndrome (Klippel-Feil syndrome, Duane syndrome and sensorineural hearing disturbance), blepharoptosis and short stature. This rare combination has not been reported previously.     

Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis: Report of six cases

The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was l-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle {S,D,L}. The ventricular septal defect was subaortic because the aorta was anterior and leftward, adjacent to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis.The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic.Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.     

Left to right shunt for congenital coronary arterio-venous fistulas

Congenital coronary artery fistulas are rare malformations that may evolve to pulmonary hypertension, heart failure and myocardial ischemia, although some may close spontaneously. Complications such as endocarditis, rupture, aneurysm or thrombosis may also be observed. Most patients are asymptomatic and the fistulas are usually detected by doppler echocardiography and angiography. We report the case of an asymptomatic 10-year-old male who was submitted because he of a heart murmur, and three coronary fistulas were diagnosed. Two originated in the left coronary artery draining into the right ventricle and the other origin was in the right coronary artery draining into the pulmonary artery trunk.     

Left Ventricular to Right Atrial Septal Defect Secondary to Blunt Thoracic Trauma Diagnosed by Transesophageal Echocardiography

A case report is presented of a 58-year-old man who developed a new holosystolic murmur 4 months after a high-speed motor vehicle accident. Cardiac catheterization demonstrated a left-to-right shunt at the right atrial level. Intraoperative transesophageal echocardiography (TEE) identified and localized a discrete atrioventricular septal defect associated with a shunt from the left ventricle to the right atrium without tricuspid regurgitation, findings that were confirmed after surgical exploration. TEE is recommended for patients with a heart murmur and a history of blunt cardiac trauma, may permit early diagnosis, and may allow surgical repair in selected patients without pre-operative catheterization. (ECHOCARDIOGRAPHY, Volume 8, May 1991)     

Atresia of the proximal right coronary artery in association with coronary artery fistula.

A 2-year-old boy presented for evaluation of a systolic murmur. Echocardiogram demonstrated dilated left main and right coronary arteries. Selective injection of the left coronary artery confirmed dilation of the left main and anterior descending coronary arteries. The left anterior descending coronary artery subsequently filled the mid portion of the right coronary artery retrograde, which emptied into a blind fistula. The orifice of the right coronary artery was atretic, with no filling from the aortic root or the fistula.