Right Heart Failure in a Patient with Critical Pulmonary Stenosis, Absent Right Pulmonary Artery, and Lung Cancer.

BACKGROUND Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiovascular malformation. More than half of UAPA cases have other cardiac lesions such as tetralogy of Fallot or septal defects. Clinical manifestations are diverse and range from heart failure after birth to an incidental finding on chest imaging during adulthood. Whereas early surgical revascularization is recommended in infancy, this is usually not feasible in the adult population. Management in these patients is aimed at treating the complications of UAPA. CASE REPORT A 67-year-old woman was evaluated for subacute right heart failure. An echocardiogram revealed pulmonary stenosis, tricuspid regurgitation, and depressed right ventricular function. Chest computed tomography (CT) showed absence of the right pulmonary artery. Additionally, there was a lung tumor in the right upper lobe. Right-heart catheterization confirmed a critically obstructed pulmonary orifice shown by hemodynamic collapse when crossing the pulmonary valve with the catheter. The patient underwent pulmonary valve balloon dilatation with right ventricular outflow tract stenting followed by percutaneous implantation of a balloon-expandable stent-valve. The clinical course was complicated by a complete heart block. Oncologic management consisted of stereotactic radiotherapy. CONCLUSIONS The combination of UAPA, pulmonary stenosis, and lung cancer is rare. Pulmonary stenosis worsens prognosis in adult patients with UAPA, but also constitutes a therapeutic target. The decision to treat the pulmonary stenosis should be based on the severity of stenosis, the degree of pulmonary hypertension, and individual anatomy. We chose percutaneous pulmonary valve implantation because our patient had a critical pulmonary stenosis with normal pulmonary pressures.

Hemothorax as an Occult Cause of Hypotension After Transseptal Puncture.

BACKGROUND Catheter ablation is an increasingly used treatment modality for arrhythmias. Periprocedural complications have a low incidence but can be life-threatening. Therefore, knowledge of possible risks during the intervention and early recognition improve patient outcomes. Transseptal puncture from the right atrium is needed for left atrial access. This procedure is a critical step that can be complicated by penetrating cardiac injury. CASE REPORT A 76-year-old patient with previous mitral valve port-access surgery underwent catheter ablation for atrial tachycardia. He developed hypotension following a challenging transseptal puncture, but transesophageal echocardiography did not demonstrate any pericardial fluid. After completing the procedure and arriving at the coronary care unit, the patient was found to be in hemorrhagic shock. CT angiography demonstrated a massive right hemothorax without active bleeding. More than 2.5 liters of blood was evacuated by chest drainage. Despite this serious complication, the patient made a full recovery without need for surgical exploration. CONCLUSIONS Hypotension during or shortly after catheter ablation should alert the physician to possible anaphylaxis, hemorrhage, or air embolism. Most patients develop bleeding near the access site or within the pericardial cavity with subsequent tamponade. This case illustrates that hemothorax due to pericardial laceration should be included in the differential diagnosis. Pleural fluid is visible on echocardiography and fluoroscopy during the procedure. Bedside lung ultrasound saves time in detecting a large hemothorax compared to CT scan. Efforts to optimize the safety of transseptal puncture remain important. Radiofrequency transseptal needles and intracardiac echocardiography are helpful tools in patients with difficult atrial septal anatomy.

Progressive Thinning of the Basal Interventricular Septum by Giant Cell Myocarditis.

We describe a patient with ventricular tachycardia and complete atrioventricular block. Remarkable thinning of the basal interventricular septum preceded left ventricular dysfunction. Endomyocardial biopsy demonstrated giant cell myocarditis. The patient received combined immunosuppressive therapy and a cardioverter-defibrillator. Eligibility screening for heart transplantation was initiated. (Level of Difficulty: Advanced.).