Giant right atrium and subvalvular pulmonary stenosis: A case report of an interesting combination.

A 20-year-old Congolese woman presented with presyncope, dyspnea, and anasarca. Past medical history was unremarkable. Echocardiography revealed a rare combination of giant right atrium (RA), a dilated and hypertrophied right ventricle, subvalvular pulmonary stenosis (subPS), severe tricuspid regurgitation (TR), pericardial effusion and what appeared to be a spontaneously closed ventricular septal defect (VSD). Cardiac Magnetic Resonance and Cardiac Computed Tomography confirmed the findings excluding the presence of intra-cardiac and extra-cardiac shunt and other associated congenital anomalies. The patient underwent subPS resection, right atrioplasty, and tricuspid annuloplasty. Multimodality approach facilitated the detection of the abnormalities and provided clarity when determining the optimal surgical strategy.

Double-orifice mitral valve repair.

We present the case of a 6-year-old patient with double-orifice mitral valve and severe regurgitation, which was successfully repaired. We demonstrate that mitral valve repair may be attempted even in complex anatomy such as the double-orifice mitral valve, where it can offer excellent results.

Infundibular pulmonary stenosis after radiotherapy for breast cancer.

Postoperative radiotherapy for breast cancer may be associated with cardiotoxicity. We present a case of acquired infundibular pulmonary stenosis that developed 43 years after radiotherapy for left breast cancer, which necessitated a right ventricular outflow tract myectomy.

Isolated subpulmonic fibrous ring, mirror-image dextrocardia and situs solitus in a young lady unreported and a near miss.

Congenital diseases causing obstruction of the right ventricular outflow tract (RVOT) are common, but the isolated subpulmonary membrane/ring is extremely rare and can be difficult to diagnose precisely, especially in adults. We report a case of surgically resected isolated subpulmonic fibrous ring in a lady with mirror-image dextrocardia and abdominal situs solitus that was misdiagnosed by echocardiography as a subaortic membrane.

LEOPARD syndrome.

LEOPARD syndrome (LS) is a rare hereditary disorder, characterised mainly by skin, facial and cardiac abnormalities. We report on the case of a six-year-old Djiboutian with typical features of LS. Multiple cardiovascular problems are described, including pulmonary infundibular, valvular and supra-valvular stenosis. A favourable course was observed after successful cardiac surgery. This is the first reported case of LS from the horn of Africa.

Surgical correction of congenital heart disease at 76 years of age.

The management of adult congenital heart disease is challenging and poses specific problems. We report a patient with ventricular septal defect and pulmonary stenosis who underwent successful repair and coronary artery bypass grafting at the age of 76 years.

Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.

Pseudoaneurysm of the right ventricle outflow tract (RVOT) is a rare complication in pediatric cardiac surgery. We report a patient who developed a right ventricular pseudoaneurysm 8 months after RVOT enlargement using a pericardial patch for infundibular pulmonary stenosis. Our patient was born with severe pulmonary valvular stenosis and treated with percutaneous balloon valvotomy in the neonatal period. Six months later, she developed infundibular pulmonary stenosis, which required surgical resection of right ventricle infundibular trabeculations and bovine pericardial patch enlargement. The postoperative period was normal. She was readmitted to hospital 5 months later complaining of wheezing, coughing and shortness of breath. Echocardiography showed a huge aneurysmal dilatation of the outflow patch in connection with the right ventricular cavity. The patient underwent resection of the pseudoaneurysm and former patch, followed by interposition of a bovine jugular vein conduit between the RVOT and pulmonary bifurcation. The early postoperative period was uncomplicated. On echocardiography, no significant residual gradient was measured through the conduit and there was no insufficiency of the valve. RVOT reconstruction with patch enlargement, homograft or conduit implantation can be the origin of pseudoaneurysms. Although their incidence is rare, they are often asymptomatic before becoming quite large and causing compression symptoms as in our patient with respiratory complaints due to airway compression. It is important to follow up these patients closely, especially in the first year after surgery since most aneurysms develop within 6 months of surgery.

Transthoracic echocardiography does not reliably predict involvement of the aortic valve in patients with a discrete subaortic shelf.

BACKGROUND: A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS: A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS: A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION: Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Long-term results of atrioventricular groove patch plasty--original method and its modification.

OBJECTIVE: Anatomically corrected malposition of great arteries (ACMGA) is associated with the leftward deviation of the proximal right coronary artery (RCA) away from the right atrioventricular (AV) groove. This anatomic feature allows a transannular subpulmonary patch plasty along the right AV groove between the RCA and the tricuspid annulus for relief of subpulmonary stenosis. This technique is also applicable to similar malformations that belong to the same spectrum. The long-term results of this original method and its modification were reviewed. METHODS: This surgical technique with a monocuspid transannular patch for subpulmonary stenosis was applied in 13 patients (seven patients with ACMGA, four patients with transposition of the great arteries after arterial switch operation (Shaher type 4 or 9) who underwent the original Konno or subaortic resection simultaneously, and two patients with discordant AV connection who have undergone conventional left ventricle to pulmonary artery repair or ventricular septation and pacemaker implantation previously). The mean age at operation was 15 years. RESULTS: Postoperative catheterisation revealed adequate relief of pulmonary stenosis, with a pressure gradient of 5.6 ± 4.5 mmHg and with normalised right ventricular pressure (35 ± 12 mmHg). There was no late death or re-operation during a postoperative follow-up period of 90 ± 58 months. All patients were in normal sinus rhythm, postoperatively, except for 3 patients (one AV block due to subaortic resection, and two pacemaker rhythms implanted previously). CONCLUSIONS: The present report describes the excellent long-term results of the AV groove patch plasty for subpulmonary stenosis in ACMGA and similar malformations by using morphological advantages. This technique provides a promising alternative to the Rastelli-type conduit repair.

Pulmonary vascular protective mechanisms in adult patients with an isolated large ventricular septal defect: a 21-year experience.

BACKGROUND: Ventricular septal defects (VSDs) are one of the most common congenital heart defects in adults. In adult patients with an anatomically large VSD and relatively preserved pulmonary vascular system, several pulmonary flow-limiting cardiac morphologic alterations (PFMA) are encountered. PATIENTS: Ninety-eight male patients (mean age 22.5 +/- 2 years) operated for an anatomically large VSD in our institution were retrospectively reviewed. PFMA in patients with an anatomically large but functionally mild-to-moderate VSD (when ratio of pulmonary to systemic flow (Q(p)/Q(s)) < 2.2 and ratio of pulmonary to systemic vascular resistance (R(p)/R(s)) < 0.3) were recorded. RESULTS: Thirty patients (31.2%) revealed a mild-to-moderate VSD in functional severity. Five PFMA were encountered in these patients: (1) ostium (os) infundibulum (n = 10, 33.3%), (2) aneurysm of the membranous septum (AMS) (n = 10, 33.3%), (3) systolic bulging of the conal septum toward the right ventricular outflow tract (n = 6, 20%), (4) prolapse of the aortic cusps (n = 2, 6.7%), and (5) attachment of the tricuspid septal leaflet to the septal crest (n = 2, 6.7%). Double-chambered right ventricle was encountered in four patients with os infundibulum and classic tetralogy-type septal malalignment in one patient with aortic cusp prolapse. Concurrent to VSD repair, resection of the os infundibulum and the AMS and aortic valve repair were performed. CONCLUSION: Presence of a large VSD and relatively preserved pulmonary vascular system in adults is associated with several PFMA. Preoperative awareness and concurrent surgical treatment of these alterations seem to be crucial to improve the expected benefit of surgical repair of VSD in this subgroup of the patients.

Autologous pericardium patch aneurysm after ventricular septal defect closure and arterial switch operation.

A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.

Infective endocarditis in the setting of infundibular-valvular pulmonary stenosis with incomplete cor triatriatum dextrum and patent foramen ovale.

Cor triatriatum dextrum is a rare congenital malformation, usually associated with complex right heart abnormalities, characterized by a membrane that divides the right atrium into two chambers. It is considered the result of the incomplete and abnormal regression of the embryonic right valve of the sinus venosus. With an incomplete regression, a fenestrated or an unfenestrated membrane may persist in the right atrium. Cor triatriatum dextrum may be seen with congenital cardiac defects associated with the right heart. We have diagnosed infective endocarditis in a 19 year old male patient with asymptomatic incomplete cor triatriatum dextrum, PFO and valvular-infundibular severe pulmonary stenosis based on the Duck criteria, with a positive blood culture and 3 minor criteria. Echocardiography did not reveal any vegetations. Antibiotherapy was given and then regions responsible of the stenosis were resected surgically. After surgery a small outlet type VSD development was observed.